ENFERMEDAD CENICIENTA O ERITEMA DISCRÓMICO PERSTANS, ACTUALIZACIÓN



Enfermedad cenicienta o discrómico perstans





ACTUALIZADO 2025




EDITORIAL ESPAÑOL:

En el año de 1.957 Oswaldo Ramírez en el primer Congreso Centro Americano de Dermatología describió por vez primera una dermatosis encontrada en su País, EL SALVADOR, con el nombre de "LOS CENICIENTOS, PROBLEMA CLÍNICO".

En 1.961 Convit y Colaboradores consideraron esta enfermedad una variante del ERITEMA PERSTANS y la denominaron ERITEMA CRÓNICO FIGURADO con MELANODERMIA.

SÍNTOMAS CLÍNICOS:

El ERITEMA DISCRÓMICO PERSTANS, se manifiesta principalmente en adultos jóvenes, y esta caracterizado por la aparición de manchas o máculas de color gris o "ceniza", (ENFERMEDAD CENICIENTA), las cuales se presentan principalmente en tórax anterior, cara , cuello y brazos, pero puede presentarse también en abdomen y piernas, incluso la membrana mucosa de la boca, sin ninguna otra sintomatología orgánica; no pican, ni molestan. En casos poco frecuentes ha sido descrita en niños.

ETIOLOGÍA:

Se dice, se especula, que la enfermedad es producida por el contacto de FERTILIZANTES utilizados en la agricultura e industria, los cuales contienen un químico denominado NITRATO de AMONIO, también ha sido relacionada con CONTRASTES RADIOLÓGICOs, PARASITOSIS, MEDICAMENTOS,  y los ANTÍGENOS DE HISTOCOMPATIBILIDAD (HLA DR4), más actualmente sigue considerándose que la causa es DESCONOCIDA.

El hecho de que esta patología ha sido observada en muchos trabajadores del campo (agricultores) es lo que hizo relacionarla con el NITRATO DE AMONIO, componente químico de los FERTILIZANTES.

TRATAMIENTO:

No existe tratamiento especifico para esta patología.se ha utilizado la CLOFAZIMINA o LAMPREN, la cual es utilizada en el tratamiento de la Lepra, porque induce hiperpigmentación de la piel y ayuda a disimular las manchas, otros elementos usados son la fotoprotección y la utilización de "fórmulas magistrales" tipo "BLANQUEADORES" para aclarar el color grisáceo de las lesiones.

La enfermedad fue aceptada como una nueva entidad nosológica en el 5to. Congreso Ibero Latino Americano de Dermatología Celebrado en Buenos Aires, ARGENTINA en el año 1963, con el nombre de ERITEMA DISCRÓMICO PERSTANS. 

En Al año de 1.967 el mismo Oswaldo Ramírez describe un estudio epidemiológico de 139 casos bajo el nombre de DERMATOSIS CENICIENTA, o ERITEMA DISCRÓMICO PERSTANS (EDP). Después de esta primera descripción comenzaron a reportarse numerosos casos en MUCHOS PAÍSES. 

En la Década de los 80 algunos autores comienzan a especular la posibilidad de que el ERITEMA DISCRÓMICO PERSTANS, es una variante del LIQUEN PLANO. Algunos lo denominaron LIQUEN PLANO PIGMENTOSO, esto en base a las siguientes características: 

SIMILITUDES:

1. El LIQUEN PLANO y el ERITEMA DISCRÓMICO PERSTANS tienen UN MISMO PATRÓN histopatológico: Una Dermatitis Liquenoide. 

2.) El liquen plano se presenta a veces como manchas grisáceas y también se presenta en personas que manipulan CONTRASTES RADIOLÓGICOS.

3.) Se describieron casos de localización clínica con patrón ZOSTERIFORME en ambas.. 

4.) Se han descrito casos de ERITEMA DISCRÓMICO PERSTANS asociados a medicamentos. Esto también ha sido descrito en el LIQUEN PLANO.

5.) El tratamiento es bastante difícil en ambos. 

En 1984. Ramírez de nuevo hace una revisión sobre todos los casos publicados y concluye que NO ES UNA ENFERMEDAD TROPICAL, sino de distribución mundial, más NO LA ASOCIA con LIQUEN PLANO. 

42 años después la controversia continua e incluso algunos autores hablan de 2 variantes de ERITEMA DISCRÓMICO PERSTANS, Otros continúan insistiendo en que debería unificarse el criterio de esta forma:

ERITEMA DISCRÓMICO PERSTANS, como una variante de LIQUEN PLANO, bajo la sinonimia de LIQUEN PLANO PIGMENTOSO. Yo estoy de acuerdo con esto 100% , pero creo que debe respetarse el NOMBRE ORIGINAL.

NOTA: Para hoy día año 2025 esta patología es reconocida MUNDIALMENTE con el nombre de ERITEMA DISCRÓMICO PERSTANS, o ENFERMEDAD "CENICIENTA", como fue propuesto en el año 1963, y su descubridor  OSWALDO RAMÍREZ del SALVADOR, Sur América.

Saludos a todos !!! 

Dr. José Lapenta R.,,, 


EDITORIAL ENGLISH:


In 1957 Oswaldo Ramírez, during the First Central-American Dermatology Congress in El Salvador; described for the first time a dermatosis found in his country, EL SALVADOR, with the name "THE ASHY,CLINICAL PROBLEM".

In 1961, Convit et al. consider this disease as a variant of ERYTHEMA PERSTANS and named it "CHRONIC FIGURED ERYTHEMA with MELANODERMA". 

CLINICAL SYMPTOMS:

ERYTHEMA DYSCHROMICUM PERSTANS primarily occurs in young adults and is characterized by the appearance of gray or "ashy" spots or macules (ASHY DISEASE). These spots appear primarily on the anterior chest, face, neck, and arms, but can also appear on the abdomen and legs, even the mucous membrane of the mouth, without any other organic symptoms; they are not itchy or bothersome. In rare cases it has been described in children.

ETIOLOGY:

It is said and speculated that the disease is caused by contact with FERTILIZERS used in agriculture and industry, which contain a chemical called AMMONIUM NITRATE. It has also been linked to RADIOLOGICAL CONTRAST AGENTS, PARASITES, DRUGS and HISTOCOMPATIBILITY ANTIGENS (HLA DR4), However, the cause is currently still considered unknown. 

The fact that this condition has been observed in many farm workers (FARMERS) is what led to its association with AMMONIUM NITRATE, a chemical component of fertilizers.

TREATMENT:

There is no specific treatment for this condition. CLOFAZIMINE or LAMPREN has been used in the treatment of leprosy because it induces skin hyperpigmentation and helps conceal the color of the spots. Other elements used include SUNSCREENS and the use of "magistral formulas" such as "BLEACHING AGENTS" to lighten the grayish color of the lesions.

This disease was accepted as new nosologic entity in the Fifth Congress of the Ibero-Latin-American College of Dermatology in Buenos Aires, ARGENTINE in  the year 1963 with the name of "ERYTHEMA DYSCHROMICUM PERSTANS" (EDP).

In To the year of 1.967 the same Oswaldo Ramirez describes an epidemiologic study of 139 cases under the name of ASHY DERMATOSIS or ERYTHEMA DYSCHROMICUM PERSTANS. After this first description they began to be reported numerous cases in MANY COUNTRIES. 

In the decade of the 80 some authors begin to speculate the probability that the ERYTHEMA DYSCHROMICUM PERSTANS is a variant of THE LICHEN PLANUS. Some denominated it LICHEN PLANUS PIGMENTOSUS, this based on the following ones characteristic: 

SIMILARITIES:

1. The LICHEN PLANUS and THE ERYTHEMA DYSCHROMICUM PERSTANS have the same histopathological picture: A Lichenoid Dermatitis. 

2.) The lichen planus is sometimes presented as ashy macules, and also occurs in people who handle RADIOLOGICAL CONTRASTS.

3.) Cases of clinical localization were described with ZOSTERIFORM pattern in both.

 4.) Cases of ERYTHEMA DYSCHROMICUM PERSTANS associated to medications have been described. This has also been described in association with LICHEN PLANUS.

5.) The treatment is quite difficult in both. 

In 1984. Ramirez again makes a revision on all the published cases and it concludes that it IS NOT A TROPICAL ILLNESS, but of world-wide distribution, but IT DOESN'T associate it with LICHEN PLANUS. 

42 years later the controversy continuous and some authors even speak of 2 variants of ERYTHEMA DYSCHROMICUM PERSTANS, Others continue insisting in that it should become unified the approach in this way:

ERYTHEMA DYSCHROMICUM PERSTANS, a variant of LICHEN PLANUS, under the synonymy of LICHEN PLANUS PIGMENTOSUS. I agree with this in 100%.but I think it should keep its ORIGINAL NAME

NOTE: For today, in the year 2025, this pathology is recognized WORLDWIDE with the name ERYTHEMA DYSCROMICUM PERSTANS or ASHY DERMATOSIS, as it was proposed in 1963, and its discoverer OSWALDO RAMÍREZ from SALVADOR, South America.

Greetings to all!!!

Dr. José Lapenta R.,,,




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****** DATA-MÉDICOS *********
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ERITEMA DISCRÓMICO PERSTANS O LIQUEN PLANO, ACTUALIZACION
ERYTHEMA DYSCROMICUM PERSTANS OR LICHRN PLANUS UPDATE
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***** DERMAGIC-EXPRESS No 73 ******* 
** 14 JUNIO 2025 / 14 JUNE 2025 ** 
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G.- Ashy Dermatosis and Lichen Planus Pigmentosus: The Histopathological Differences (2019). 

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1.) "Los cenicientos. Problema Clinico". (The Ashy. Clinical problem) 

2.) "Eritema Discromico Perstans" (Eritema figurado cronico con melanodermia).(Chronic Figured Erythema with Melanodermia) 

3.) Sobre la Etiopatogenia del eritema discrómico perstans (Dermatosis Cenicienta). Posibilidad de una Melanosis Neuro-cutánea 

4.) The ashy dermatosis (erythema dyschromicum perstans) 

5.) Ashy dermatosis or lichen planus pigmentosus: what is in a name? 

6.) ERYTHEMA DYSCHROMICUM PERSTANS (ASHY DERMATOSIS) THE ENTITY 

7.) [Ashy dermatitis. Comments on 2 clinical forms]. 

8.) Unilateral ashy dermatosis occurring in a child. 

9.) [Current status of ashy dermatosis. Synonym--erythema dyschromicum perstans]. 

10.) [Ashy-dermatosis--a case with spontaneous disappearance of the cutaneous lesions]. 

11.) Erythema dyschromicum perstans and lichen planus. 

12.) Ashy dermatosis. An apoptotic disease? 

13.) Ashy dermatosis--a variant of lichen planus? 

14.) Erythema dyschromicum perstans in early childhood. 

15.) Fixed drug eruption presenting as erythema dyschromicum perstans: a flare without taking any medications. 

16.) Reticulate postinflammatory hyperpigmentation with band-like mucin deposition. 

17.) Erythema dyschromicum perstans: report of a new case and critical review of the literature. 

18.) Involvement of cell adhesion and activation molecules in the pathogenesis of erythema dyschromicum perstans (ashy dermatitis). The effect of clofazimine therapy. 

19.) Periorbital hyperpigmentation and erythema dyschromicum perstans. 

20.) Erythema dyschromicum perstans and lichen planus: are they related? 

21.) Erythema dyschromicum perstans. Immunopathologic studies. 

22.) Simultaneously active lesions of vitiligo and erythema dyschromicum perstans. 

23.) Mononuclear cell subpopulations and infiltrating lymphocytes in erythema dyschromicum perstans and vitiligo. 

24.) Chlorothalonil, a possible cause of erythema dyschromicum perstans (ashy dermatitis). 

25.) Lichen planus pigmentosus presenting in zosteriform pattern. 

26.) [Erythema dyschromicum perstans versus lichen planus]. 

27.) Erythema dyschromicum perstans. 

28.) Erythema dyschromicum perstans. 

29.) [Erythema cinitiensis perstans]. 

30.) [Idiopathic eruptive macular pigmentation]. 

31.) [Erythema dyschromicum perstans]. 

32.) Erythema dyschromicum perstans. A follow-up study from northern Finand. 

33.) Ingestion of ammonium nitrate as a possible cause of erythema dyschromicum perstans (ashy dermatosis). 

34.) [Erythema dischromicum perstans (ashy dermatosis). Report of two cases]. Pathologica 1993 Sep-Oct;85(1099):533-41 

35.) [Ashy dermatosis]. 

36.) [Ashy dermatosis. Review of the literature and report of a case]. 

37.) [Ashy dermatosis (erithema dyschromicum perstans): prospective study of 23 patients]. 

38.) Reticulate, patchy and mottled pigmentation of the neck. Acquired forms. 

39.) Lichen planus pigmentosus associated with acrokeratosis of Bazex. 

40.) Ashy dermatosis and lichen planus pigmentosus: a clinicopathologic study of 31 cases. 

41.) [Lichen ruber exanthematicus et pigmentosus in mercury poisoning. A contribution to individual pathology in occupational medicine]. 

42.) [Lichen planus and lichen planus pigmentosus following gold therapy--case reports and review of the literature]. 

43.) Lichen planus pigmentosus of the oral mucosa: a rare clinical variety. 

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1.) "Los cenicientos. Problema Clinico". (The Ashy. Clinical problem) Memoria del Primer Congreso Centro Americano de Dermatologia, San salvador, 

1957:122-130 Ramirez, C.O 

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2.) "Eritema Discromico Perstans" (Eritema figurado cronico con melanodermia).(Chronic Figured Erythema with Melanodermia) 

Dermat. Venezolana, 1961, 2:118-164 

Convit, J., Kerdel-Vegas, F., Rodriguez, G. 

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3.) Sobre la Etiopatogenia del eritema discrómico perstans (Dermatosis Cenicienta). Posibilidad de una Melanosis Neuro-cutánea Derm Venez 1995; 

33:149-151 

Homez-Chacin, 1. Barroso T. 

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4.) The ashy dermatosis (erythema dyschromicum perstans): epidemiological study and report of 139 cases. 

Ramirez C.O. Cutis 1967; 3:244-7 

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5.) Ashy dermatosis or lichen planus pigmentosus: what is in a name? 

Arch Dermatol 1986 Feb;122(2):133 

Bhutani LK 

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6.) ERYTHEMA DYSCHROMICUM PERSTANS (ASHY DERMATOSIS) THE ENTITY 

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SOURCE: Sobre la Etiopatogenia del eritema discrómico perstans (Dermatosis Cenicienta). Posibilidad de una Melanosis Neuro-cutánea 

Derm Venez 1995;33:149-151 Homez-Chacin, 1. Barroso T. 


ABSTRACT 


In 1957 Oswaldo Ramírez, during the First Central-American DermatoIogy  Congress in El Salvador; described for the first time a dermatosis found in  his country wjth the name "The Ashy. Clinical problem". In 1961, Convit et  al. consider this disease as a variant ot erythema perstans and name it  "Chronic Figured Erythema with Melanodermia". this disease was accepted as  new nosologic entity in the Fifth Congress of the Ibero-Latin-American  College of Dermatology in Buenos Aires in 1963 with the name of "Erythema Dyschromicum Perstans". 


According to O. Ramírez. "Erythema Discromicum Perstans (Ashy Dermatosis)  is a chronic skin disease characterized by typical and marked changes in  color of the skin (hyperpigmented macules, black-board gray, variable), of  slow evolution, persistent, with no concomitant local alterations, but  which sometimes have repercussions on the psychic state of the patient,  according to the size of the lesion. It is not accompanied by any other  cutaneous or symptomatic abnormality. It is not influenced by climate,  racial, dietary or occupational factors". This dermatosis can appear at any  age, from one to eighty years. Either sex can be equally affected, even  though it is more frequent in females. lnitially lesions are localized,  but they can become generalized, not involving scalp, annexes or  palmo-plantar regions. 


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7.) [Ashy dermatitis. Comments on 2 clinical forms]. 

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Med Cutan Ibero Lat Am 1986;14(2):95-9 


Carvajal Huerta L, Uraga Pazmino E, Loayza Vivanzo E, Sabando Sanchez R,  Garcia Atiaga I, Jeny E 


The typical clinical characteristic of the ashy dermatitis is the fact that  in case of light colored skin, the grey colour remains invariable. There is  a new clinical form which is called the Brown Cinder Dermatitis which is  characterized by a disciplined localization in the center of the face,  trunk or upper extremities together with an inexorable change of the grey  colour at the beginning to a brown colour at the end on the other it, has  be enhanced the existence of the nummular form which is characterized by  the existence of many greyish independent spots of circular or oval form  mainly at the level of the trunk. 


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8.) Unilateral ashy dermatosis occurring in a child. 

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Arch Dermatol 1984 Nov;120(11):1491-3 


Urano-Suehisa S, Tagami H, Iwatsuki K 


An unusual case of ashy dermatosis of Ramirez occurred in a 5-year-old-girl  who had ash-colored hyperpigmented macules and plaques on the left leg and  the left side of the trunk. These unilateral lesions showed histopathologic  changes of a lichenoid tissue reaction. Although the exact cause of ashy  dermatosis remains unknown, the positive test result for serum rheumatoid  factor and the granular deposition of IgM at the dermoepidermal junction  found in our case has immunologic implications. 


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9.) [Current status of ashy dermatosis. Synonym--erythema dyschromicum perstans]. 

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Med Cutan Ibero Lat Am 1984;12(1):11-8 

[Article in Spanish] 


Ramirez O, Lopez Lino DG 


A clinical, histopathological, therapeutical and epidemiologic review of  Ashy Dermatosis is done. This research has been taken from scientific  publications, computers and personal communications. In twenty five years  of scientific medical investigations it has not been determined its truth  etiology , nor its therapeutic; this confirms it, as a new nosologic entity  well defined.


The epidemiologic researches are concludent that this is not  a tropical disease exclusively, but one of all over the world. We hope to  obtain more references on this dermatosis throughout the Sessions of 

Specialties of CILAD regarding this disease. 


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10.) [Ashy-dermatosis--a case with spontaneous disappearance of the cutaneous  lesions]. 

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Z Hautkr 1983 Jan 15;58(2):113-20 

[Article in German] 


Schneider I, Varga M, Zombai E, Husz S 


A 14-years-old girl suddenly developed typical dermal signs of  ashy-dermatosis at the time of menarche. After one year, the signs began to  fade and had disappeared completely two years later. The direct  immunofluorescence and the ultrastructure resemble lichen planus. Endocrine  factors may have played a part in the presented case, considering the  sudden onset at the time of menarche. 


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11.) Erythema dyschromicum perstans and lichen planus. 

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Arch Dermatol 1982 Sep;118(9):683-5 


Naidorf KF, Cohen SR 


Erythema dyschromicum perstans (EDP) is a cutaneous pigmentary disturbance  originally considered to be a variant of erythema perstans. The nosologic  identity of EDP has been challenged repeatedly as the number of patients  with concurrent EDP-like eruptions and lichenoid disorders has increased.  In this report, we describe a woman who had EDP for two years before the  onset of classic lichen planus (LP).


The active lesions of LP gradually  evolved into typical ashy-gray macules of EDP. This case provides further  support for the concept that EDP and EDP-like conditions should be  classified, in certain instances, as erythema dyschromicum variants of LP. 


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12.) Ashy dermatosis. An apoptotic disease? 

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Arch Dermatol 1981 Nov;117(11):701-4 


Person JR, Rogers RS 3d 


Of nine patients with ashy dermatosis, eight were women; most were young  adults when the dermatosis began. The incidences of atopy (five patients)  and thyroid disease (three patients) were striking.


Although some of the  patients were clinically atypical, histologic study in all cases showed  basal cell vacuolation, Civatte bodies, pigmentary incontinence, and a mild  perivascular lymphohistiocytic infiltrate. Direct immunofluorescence  microscopy, performed in four cases, showed igM cytoid bodies. We  hypothesize that the postinflammatory hyperpigmentation in ashy dermatosis  and, perhaps, in other dermatoses may occur on the basis of basal cell 

apoptosis. 


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13.) Ashy dermatosis--a variant of lichen planus? 

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Cutis 1980 Jun;25(6):631-3 


Kark EC, Litt JZ 


Immunofluorescence studies on a patient with clinical and histological  evidence of ashy dermatosis revealed a pattern commonly associated with  lichen planus. In view of these findings, the possibility of a relationship  between ashy dermatosis and lichen planus is suggested. 


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14.) Erythema dyschromicum perstans in early childhood.  J Dermatol 1999 Feb;26(2):119-21 

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Lee SJ, Chung KY 

Department of Dermatology, Yonsei University College of Medicine, Seoul,  Korea. 


Erythema dyschromicum perstans (EDP) is a rare disorder characterized by  asymptomatic, slowly progressive, ash-gray macular pigmentation of the skin  which usually occurs from age 5 through adult life.


We have experienced two  cases of EDP in children aged 2 and 3, both exceptionally younger than the  previously reported cases. We therefore suggest that EDP should be included  in the differential diagnosis of pigmentary disorders occurring at an early  age. 


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15.) Fixed drug eruption presenting as erythema dyschromicum perstans: a flare  without taking any medications. 

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Dermatology 1998;197(4):383-5 


Mizukawa Y, Shiohara T 

Department of Dermatology, Kyorin University School of Medicine, Tokyo,  Japan. 


Fixed drug eruption (FDE) can present as multiple pigmented macules that  flare at fixed sites even when the patient has taken no medications.  Although this presentation is not characteristic of FDE, it must be borne  in mind in order to make a correct diagnosis. We describe such a patient  whose condition was initially diagnosed as erythema dyschromicum perstans  (EDP). Immunohistochemically intraepidermal T cells were distributed  between basal and suprabasal keratinocytes in the lesional skin, a finding  suggestive of FDE.


A flare occurred not only with exposure to theophylline  but also without exposure. A flare has never recurred and pigmented macules  faded gradually after avoiding theophylline. On the basis of these  findings, we recommend that patients with an EDP-like presentation be  examined completely for causes such as drugs before labeling the cutaneous  lesions. 


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16.) Reticulate postinflammatory hyperpigmentation with band-like mucin deposition. 

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Int J Dermatol 1998 Nov;37(11):829-32 


Noto G, Pravata G, Arico M 

Department of Dermatology, University of Palermo, Policlinico P. Giaccone,  Italy. 


BACKGROUND: Mucinoses of the skin are a group of disorders sharing  accumulation of mucin in the skin or hair follicles. Postinflammatory  hyperpigmentation, with pigmentary incontinence, is due to loss of melanin  from epidermal basal cells and its accumulation in dermal macrophages.  METHODS: We describe clinicopathologic features of two patients presenting  with the association of pigmentary incontinence with an unusual diffuse,  band-like dermal deposition of mucin, clinically presenting with reticular  pigmented macular lesions.


RESULTS: Two patients were observed with  asymptomatic, persistent, reticular, pigmented patches located in the  flexures, thighs, neck and back. Histology showed melanophages with a  diffuse, band-like dermal deposition of mucin, an increased number of  fibroblasts, a slight T-cell infiltrate and scattered mast cells. Blood  markers of lupus erythematosus were negative.


CONCLUSIONS: These findings  may draw attention to pigmentary disorders such as lichen planus  pigmentosus, erythema dyschromicum perstans, pigmentatio maculos eruptiva  idiopathica, dermatopathia pigmentosa reticularis, prurigo pigmentosa and  frictional melanosis.


None of these entities, however, includes mucin  deposition among its microscopic features. Macules were not preceded by  erythema or any other lesions. We suggest that our cases could belong to  group II of Rongioletti and Rebora's classification, i.e. they could be  cases of secondary mucin deposition in postinflammatory hyperpigmentation,  possibly in an unusual form of lichen planus pigmentosus or, less likely,  frictional melanosis. 


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17.) Erythema dyschromicum perstans: report of a new case and critical review of  the literature. 

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J Dermatol 1998 Nov;25(11):747-53 


Combemale P, Faisant M, Guennoc B, Dupin M, Heyraud JD 

Department of Dermatology, Military Hospital Desgenettes, Lyon, France. 


Erythema dyschromicum perstans (EDP), described by Convit et al. in 1961,  is a rare dermatosis. Its relationship with ashy dermatosis (AD), described  by Ramirez in 1957, is still a matter of debate.


We report a typical case  of EDP. The patient, of North African origin, had a dyschromic (hypo- and  hyperpigmented) eruption on the chest and limbs for 2 years. The lesions  were occasionally surrounded by a papular border which spread slowly and  centrifugally. Histological examination showed a lichenoid infiltrate. A  carcinoma of the lung was simultaneously discovered.


No treatment was  given, EDP is infrequent and often considered identical to ashy dermatosis  in the literature. However, the clinical aspects of the two diseases  differ. The main features of these two diseases are reviewed and compared  on the basis of a literature review. We conclude that EDP and AD are  distinct clinical entities. 


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18.) Involvement of cell adhesion and activation molecules in the pathogenesis  of erythema dyschromicum perstans (ashy dermatitis). The effect of  clofazimine therapy. 

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Arch Dermatol 1997 Mar;133(3):325-9 


Baranda L, Torres-Alvarez B, Cortes-Franco R, Moncada B, Portales-Perez DP,  Gonzalez-Amaro R 

Department of Immunology, School of Medicine, University of San Luis  Potosi, Mexico City, Mexico. 


OBJECTIVES: To assess the expression of several cell adhesion and  lymphocyte activation molecules in erythema dyschromicum perstans lesions,  and to evaluate the effect of clofazimine therapy on the expression of  these molecules.


DESIGN AND METHODS: A prospective study. Skin biopsy  samples were obtained from patients before and after 3 months of  clofazimine therapy, and the expression of cell adhesion and activation  molecules was assessed by an immunohistochemical technique.


SETTING: This  study was performed in a clinical referral center and an immunology  research laboratory.


PATIENTS: We studied 6 patients with erythema  dyschromicum perstans. A diagnosis was made on the basis of clinical and  histological criteria. Two patients discontinued participation in the  study: one because of adverse effects and the other for unknown reasons. 


INTERVENTIONS: Patients were treated with clofazimine, 100 mg/d, for 3  months.


MAIN OUTCOME MEASURES: Expression of cell adhesion and lymphocyte  activation molecules in skin biopsy specimens before and after clofazimine  therapy. RESULTS: Before clofazimine therapy, we detected a noticeable  expression of intercellular adhesion molecule 1 and major  histocompatibility complex class II molecules (HLA-DR) in the keratinocyte  basal cell layer. In addition, CD36, a thrombospondin receptor that is not  expressed by normal skin, was detected in the strata spinosum and  granulosum. The dermal cell infiltrate expressed the activation molecule  AIM/CD69 and the cytotoxic cell marker CD94. After clofazimine therapy, the  expression of intercellular adhesion molecule 1 and HLA-DR disappeared, as  well as the mononuclear cell infiltrate.


CONCLUSIONS: Our results suggest  that some cell adhesion and activation molecules are involved in the  pathogenesis of erythema dyschromicum perstans. Clofazimine appears to have  an important effect on the inflammatory phenomenon of erythema dyschromicum  perstans. 


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19.) Periorbital hyperpigmentation and erythema dyschromicum perstans. 

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Can J Ophthalmol 1992 Dec;27(7):353-5 


Ing EB, Buncic JR, Weiser BA, de Nanassy J, Boxall L 

University of Toronto, ON. 



Erythema dyschromicum perstans is a rare idiopathic dermatosis  characterized by ash-grey, well-demarcated skin lesions, which may involve  the face.


We describe an 8-year-old girl with erythema dyschromicum  perstans presenting as bilateral acquired periorbital hyperpigmentation.  The changes seen on histologic study of a skin biopsy specimen were  consistent with the clinical diagnosis. The various causes of periorbital  hyperpigmentation and characteristics of erythema dyschromicum perstans are  reviewed. 


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20.) Erythema dyschromicum perstans and lichen planus: are they related? 

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J Am Acad Dermatol 1989 Aug;21(2 Pt 2):438-42 


Berger RS, Hayes TJ, Dixon SL 

Wilford Hall USAF Medical Center, Lackland Air Force Base, Texas. 


A 53-year-old woman initially had lichen planus primarily on her  extremities. Approximately 1 year later, lesions consistent with erythema  dyschromicum perstans were observed. Both diseases cleared with  griseofulvin therapy but returned after discontinuation of the drug.  Retreatment with griseofulvin again resulted in clearing. 


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21.) Erythema dyschromicum perstans. Immunopathologic studies. 

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J Am Acad Dermatol 1989 May;20(5 Pt 2):882-6 


Miyagawa S, Komatsu M, Okuchi T, Shirai T, Sakamoto K 

Department of Dermatology, Nara Medical University, Japan. 


Immunopathologic studies of a patient with clinical and histologic evidence  of erythema dyschromicum perstans revealed Ia antigen expression on  epidermal keratinocytes, pronounced OKT4 and OKT6 staining of epidermal  dendritic cells, and dermal infiltration of T lymphocytes of both  helper-inducer (OKT4) and suppressor-cytotoxic (OKT8) phenotypes--a pattern  commonly associated with lichen planus. These findings, taken in  conjunction with positive IgG staining on colloid bodies, suggest the  possibility that erythema dyschromicum perstans and lichen planus have  similar disease processes. 


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22.) Simultaneously active lesions of vitiligo and erythema dyschromicum perstans. 

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Arch Dermatol 1988 Aug;124(8):1258-60 


Henderson CD, Tschen JA, Schaefer DG 

Departments of Pathology, Baylor College of Medicine, Houston, Tex. 


Recently, a patient presented to us with skin that had areas of normal  pigmentation, hyperpigmentation, and depigmentation. Workup eventually  showed him to have simultaneously active lesions of a depigmenting  disorder, vitiligo, and a hyperpigmenting disorder, erythema dyschromicum  perstans. 


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23.) Mononuclear cell subpopulations and infiltrating lymphocytes in erythema  dyschromicum perstans and vitiligo. 

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Histol Histopathol 1987 Jul;2(3):277-83 


Gross A, Tapia FJ, Mosca W, Perez RM, Briceno L, Henriquez JJ, Convit J 

Institut of Biomedicine, Caracas, Venezuela. 


Erythema dyschromicum perstans (EDP) and vitiligo are two cutaneous  pigmentary dermatoses of unknown etiology. In the present study, the  leukocyte infiltrates in the affected skin of EDP and vitiligo patients  were studied using the avidin-biotin (ABC) immunoperoxidase technique and  monoclonal antibodies which recognise the following mononuclear cell  subgroups: T-suppressor/cytotoxic (CD8-Leu-2), T-helper (CD4 = OKT4),  T-suppressor + macrophages (Leu-15), Pan T (CD3 = Leu-4), macrophages  (Leu-M3) and Langerhans cells (CD1 = Leu-6), and other cellular markers  such as Ia antigens and the Interleukin-2 receptor (CD25 = TAC).


The  immunocytochemical analysis showed a selective accumulation of CD3+, CD8+,  Leu-15-, T-cytotoxic cells in the epidermis of both EDP and early lesions  of vitiligo.


In addition, an increase in the number of epidermal Langerhans  cells (CD1+) was observed in some cases of EDP and vitiligo. The CD4/CD8  ratios in affected and uninvolved skin for both disorders were not  significantly different, although values lower than unity were only  observed in the infiltrates of affected skin. Ia antigen positivity was  observed in the dendritic cells of the dermis and epidermis, as well as in  most of the lymphoid cells within the infiltrates for both diseases.  Macrophages (Leu-M3) in EDP dermal infiltrates were generally found  adjacent to extracellular melanin pigment.


Lymphocytes expressing TAC  (CD25) surface antigens were also present in the dermal infiltrates. These  morphological observations suggest a possible immune cell participation in  the dyschromia of such cutaneous disorders. 


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24.) Chlorothalonil, a possible cause of erythema dyschromicum perstans (  ashy dermatitis). 

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Penagos H; Jimenez V; Fallas V; O'Malley M; Maibach HI 

Occupational Dermatology Service, Social Security Hospital, Panama. 

Contact Dermatitis (DENMARK) Oct 1996 35 (4) p214-8 ISSN: 0105-1873 

Language: ENGLISH 

Document Type: JOURNAL ARTICLE 

Journal Announcement: 9705 


Subfile: INDEX MEDICUS 


We studied 39 patients with erythema-dyschromicum-perstans-like  dermatitis seen at Changuinola Hospital in Panama. They were compared  with 41 controls.


The 2 groups were native field workers of the banana  plantations exposed to many pesticides. In 34 patients, there was a  positive patch test reaction to 2,4,5,6-1,3-  tetrachloroisophthalonilnitrile (chlorothalonil, TCPN) 0.001% in acetone.  In 39 cases, biopsies showed a lichenoid tissue reaction compatible with a  chronic pigmented dermatitis or erythema-dyschromicum-perstans-like  dermatitis.


Chlorothalonil is possibly the cause of the pigmented  dermatitis observed in the 39 banana farm workers studied. Until  additional studies are carried out, we consider this a possible rather  than definite cause-and-effect relationship. 


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25.) Lichen planus pigmentosus presenting in zosteriform pattern. 

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Cho S; Whang KK 

Department of Dermatology, College of Medicine, Ewha Womans University, 

Seoul, Korea. 

J Dermatol (JAPAN) Mar 1997 24 (3) p193-7 ISSN: 0385-2407 

Language: ENGLISH 

Document Type: JOURNAL ARTICLE 

Journal Announcement: 9709 


Subfile: INDEX MEDICUS 


Lichen planus pigmentosus (LPP) has thus far been described as a  condition of unknown etiology which clinically differs from the classical  lichen planus (LP) by exhibiting dark brown macules and/or papules mostly  in exposed areas and flexural folds and a longer clinical course without  pruritus or scalp, nail or mucosal involvement.


Histopathologically, LPP  shows the typical changes seen in LP, but with thinning of epidermis. We  report a case of LPP that developed in a unilateral, zosteriform pattern  on the left flank of a 49-year-old man.


This case seems to lie in the  middle of the spectrum between classical LP and ashy dermatosis, and, to  the best of our knowledge, is the first report of LPP presenting in the  zosteriform pattern. 


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26.) [Erythema dyschromicum perstans versus lichen planus]. 

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Med Cutan Ibero Lat Am 1987;15(1):89-92 


Leonforte JL, Pelaez de di Bari O 


Erythema dyschromicum perstans and lichen planus have been regarded as  related. We describe the case of a 41 year old man presenting a relapsing  dermatitis consisting of erythematous patches leaving behind ashy-gray  macules. The lesions of lichen planus were atypical and the ashy dermatosis  was transient. The possibility that some cases of lichen planus lead to an  erythema dyschromicum perstans is discussed. 


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27.) Erythema dyschromicum perstans. 

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Cutis 1986 Jan;37(1):42-4 


Lambert WC, Schwartz RA, Hamilton GB 


A 29-year-old woman from Trinidad experienced the rapid onset of extensive  lesions characteristic of erythema dyschromicum perstans following an x-ray  study using orally administered contrast.


Eleven months later a skin biopsy  specimen showed few epidermal changes but extensive incontinence of melanin  pigment and marked dilatation of lymphatics in the superficial dermis.  Results of a systemic evaluation were normal. The available data on this  disease are analyzed and conclusions offered regarding its nature and causes. 


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28.) Erythema dyschromicum perstans. 

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J Am Acad Dermatol 1980 Apr;2(4):295-302 


Tschen JA, Tschen EA, McGavran MH 


Erythema dyschromicum perstans (EDP) was first described in 1957, and  electron microscopic studies were reported in 1969. Herein, we describe  five cases and compare light and electron microscopic findings, direct and  indirect immunofluorescence, and dopa-positive melanocyte counts between  normal and affected skin.


The results indicate that EDP is a clinically  characteristic disorder with a lichenoid reaction in its active phase. This  lichenoid reaction leads to a pronounced incontinence of pigment and to  decreased numbers of melanocytes and of tyrosinase activity in the involved  epidermis. These findings support the suggestion that EDP and lichen planus  pigmentosus are possible the same entity.


Direct inmunofluorescence and  fine structural studies show similar findings to lichen plunus. Patients  have low-titer antibodies to extranuclear basal cell components. 


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29.) [Erythema cinitiensis perstans]. 

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Med Cutan Ibero Lat Am 1980;8(1-3):11-4 


Sittart JA, Tayah M 


The authors report a case of Erythema Cinitiensis Perstans, in a thirty-one  years old white man. This dermatosis, reported in the literature also es  Ashy Dermatitis and Erythema Dyschromicum Perstans, is here in Brazil  exceptional. Clinically this dermatosis is characterized by the presence of  gray patches in a sometimes surrounded by an erythematous active border.  The lesions may occur on any area of the integument, sparing the hair  scalp, palms, soles and nails.


The therapy has been ineffective in all the  cases, but one reported by Stevenson and Miura and in our case, where the  patients have improved after Dithiazanine iodide treatment due to  intestinal parasitoses by the Tricocephalus trichiurus. 


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30.) [Idiopathic eruptive macular pigmentation]. 

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Ann Dermatol Venereol 1978 Feb;105(2):177-82 PORTUGAL 


Degos R, Civatte J, Belaich S 


The feeling of the authors is that their seven reported cases of a  pigmented dermatosis are different from the ashy dermatosis and from the  erythema dyschromicum perstans.


This disease, which affects children and  teenagers, males as well as females, is characterized by pigmented macules  5-25 mm in diameter, affecting the neck, the trunk and the limbs. The first  symptom is whether a pigmented spot, or an erythematous, papular or  achromic lesion; in the latter instance the pigmentation occurs only  secundarily. In most of the cases this dermatose is slowly and  spontaneously regressive.


The histological picture is not really specific.  In one case there was a marked intraepidermal dyskeratosis of the sweat  duct openings. The etiology remains unknown. 


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31.) [Erythema dyschromicum perstans]. 

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Hautarzt 1977 Oct;28(10):539-41 


Moller-Vietheer M, Goos M 


The case of a 4 1/2 year old girl with the typical clinical picture of  erythema dyschromicum perstans is presented. A survey of the literature on  this dermatosis is described. 


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32.) Erythema dyschromicum perstans. A follow-up study from northern Finand. 

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Dermatologica 1977;155(1):40-4 


Palatsi R 


Four patients with ashy dermatosis are described. Their ages varied from 8  to 12 years. Three had typical widespread macular eruptions and one had a  linear lesion not described before. The follow-up investigation revealed  that the eruption disappeared within 2 years in three of the patients. One  patient could not be followed. The duration of the disease and the  linearity of one lesion resembled lichen ruber planus. 


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33.) Ingestion of ammonium nitrate as a possible cause of erythema dyschromicum  perstans (ashy dermatosis). 

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Dermatologica 1975;150(5):287-91 


Jablonska S 


Clinically and histologically typical erythema dyschromicum perstans has  been provoked by repeated ingestion of very small amounts of a fertilizer,  ammonium nitrate.


It has been used by a boy who has noticed accidentally  that after licking the fertilizer he developed hyperpigmentations. His main  purpose was to avoid school attendance, and he developed a very ingenious  method of licking the fertilizer once in several weeks in such a way that  it did not produce any digestive or other troubles.


His brother, an  uniovular twin who had no contact with ammonium nitrate, was healthy. The  case supports Pinkus' hypothesis that this type of lesions may be related  to some environmental contaminant. Attention is called to a possible food  contamination by ammonium nitrate. 


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34.) [Erythema dischromicum perstans (ashy dermatosis). Report of two cases].  Pathologica 1993 Sep-Oct;85(1099):533-41 

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[Article in Italian] 


Paradisi M, Mostaccioli S, Celano G, Angelo C, Ruatti P, Ferranti G, Onetti 

Muda A, Faraggiana T 

IV Divisione e Dermatologia Pediatrica, IDI-IRCCS, Roma. 


Clinical, histological and ultrastructural investigations of two cases of  Erythema Dischromicum Perstans (EDP) are reported. EDP is a chronic  pigmented lesion of the skin, and its etiology is still unknown. The  reported cases showed clinical and ultrastructural differences from what  already described in the literature.


EDP is also difficult to differentiate  from other cutaneous pigmented lesions: clinical and morphologic  differences and/or similarities are therefore discussed and compared. The  usefulness for a correct diagnosis of the co-existence of optical and  ultrastructural lesions which are not pathognomonic per se, is also stressed. 


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35.) [Ashy dermatosis]. 

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Vestn Dermatol Venerol 1989;(11):57-8 

[Article in Russian] 


Kasimov N, Kiiamov FA, Naimova MR 


Five patients with ashy dermatitis are described, aged 6 to 14, one boy and  four girls. The process is characterized by dirty grayish or  ash-gray-colored maculae on the skin of the trunk and limbs.


These maculae  do not tend to fuse, nor exfoliate; they are not associated with any  subjective sensations. Thorough examinations have not revealed any  osteomuscular or visceral abnormalities.


The disease runs a benign course,  and only the cosmetic defect worries the patients. 


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36.) [Ashy dermatosis. Review of the literature and report of a case]. 

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Med Cutan Ibero Lat Am 1988;16(1):31-6 

[Article in Portugese] 


de Azevedo LM, Porto JA 

Servico de Dermatologia do Hospital Universitario Pedro Ernesto, da  Universidade do Estado do Rio de Janeiro. 


The authors make a review of the epidemiologic, etiopathogenic, clinical,  laboratory and therapeutic aspects of ashy dermatosis, described in 1957 by  Ramirez in El Salvador, of which more than 150 cases have been described up  to now in different continents. The disease is exclusively cutaneous,  presents peculiar clinical features with a lichenoid tissue reaction, and  has no specific treatment or known etiology.


The authors report a case in a  Brasilian man, followed up for three years. The treatment of a T. trichiura  infestation did not change the cutaneous features, as opposed to what has  been reported. 


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37.) [Ashy dermatosis (erithema dyschromicum perstans): prospective study of 23  patients]. 

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Med Cutan Ibero Lat Am 1988;16(5):407-12 

[Article in Spanish] 


Navarro Jimenez BR, Sanchez Navarro LM 

Servicio de Dermatologia, Universidad Autonoma, Benito Juarez, Oaxaca,  Mexico. 


During the period between january 1984 to july 1987 in the Hospital Unit  "Presidente Juarez" ISSSTE, Oaxaca (Mexico), for the first time was given  dermatology consult to 2,683 patients among them, 23 who had ashy  dermatosis (Erithema dyschromicum perstans), a study was done for the  present time and the future of the same, discarding it as possible cause of  that nosological entity: treponemal disease, parasitic, infectious, hepatic  and renal.


It is commented in relation with the ashy dermatosis and lichen  planus, the clinicals variants and the possible sun etiology. 


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38.) Reticulate, patchy and mottled pigmentation of the neck. Acquired forms. 

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Dermatology 1998;197(3):291-6 


Lautenschlager S, Itin PH 

Outpatient Clinic of Dermatology, Triemli Hospital, Zurich, Switzerland. 


Besides the inherited forms of mottled and reticulate pigmentation, a vast  number of diseases and trigger mechanisms can lead to acquired pigmentation  of the neck. Nonhereditary variants of reticulate and mottled pigmentation  can affect the neck as a typical site and therefore may give a diagnostic  clue or it can occur sporadically on the neck as well as on other sites. A  well-known and important factor in the pathogenesis is exposure to  sunlight.


Sun-induced pigmentation often presents on the neck and may  result from phototoxic, photoallergic and cumulative actinic damage.  Frequent forms comprise berloque dermatitis, Riehl's melanosis,  poikiloderma of Civatte and tanning bed lentigines. Different infections  may also lead to this distinct skin alteration as pediculosis capitis,  pityriasis versicolor and syphilis II.


Treatment-induced irregular  pigmentations may occur after applications of topical agents (e.g.  diphenylcyclopropenone), systemic medication (e.g. 5-fluorouracil,  chlorpromazine), as a complication of laser resurfacing or as a chronic  graft-versus-host reaction. Different neoplasms may also involve the neck.  Widespread pigmented basal cell carcinoma, cutaneous T-cell lymphoma,  syringolymphoid hyperplasia and histiocytic diseases may lead to  reticulated pigmentation.


Various other infrequent conditions as connective  tissue diseases, malnutrition, lichen planus pigmentosus and others are  summarized. The neck, a readily accessible site to medical inspection, may  have an underestimated value for the diagnosis of different skin diseases. 


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39.) Lichen planus pigmentosus associated with acrokeratosis of Bazex. 

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Clin Exp Dermatol 1994 Jan;19(1):70-3 


Sassolas B, Zagnoli A, Leroy JP, Guillet G 

Department of Dermatology, C.H.U. Brest, France. 


A patient with an acquired pigmentation related to lichen planus  pigmentosus is described. Features of acrokeratosis of Bazex were  associated, related to a head and neck carcinoma. Both cutaneous conditions  disappeared after treatment of the neoplasia. Diagnostic criteria of lichen  planus pigmentosus are reviewed. The paraneoplastic nature of this original  observation of lichen planus pigmentosus is discussed. 


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40.) Ashy dermatosis and lichen planus pigmentosus: a clinicopathologic study of  31 cases. 

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Int J Dermatol 1992 Feb;31(2):90-4 


Vega ME, Waxtein L, Arenas R, Hojyo T, Dominguez-Soto L 

Department of Dermatology, Hospital General Dr. Manuel Gea Gonzalez, Mexico  City, Mexico. 


The clinical and histopathologic characteristics of patients with ashy  dermatosis (n = 20) and lichen planus pigmentosus (n = 11) were analyzed.  We found significant clinical differences between both dermatoses,  supporting our opinion that they are two separate conditions. Both  dermatoses were histologically similar. 


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41.) [Lichen ruber exanthematicus et pigmentosus in mercury poisoning. A  contribution to individual pathology in occupational medicine]. 

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Z Hautkr 1990 Nov;65(11):1013-8, 1021 

[Article in German] 


Marsch WC, Groebe G 

Zentrum der Dermatologie und Venerologie, Abteilung I, Johann Wolfgang  Goethe-Universitat, Frankfurt am Main. 


We report on a 21-year-old man professionally exposed to mercury, who  developed lichen planus. This case must be regarded as a dispositional  reaction and is in Germany entitled to indemnification in terms of a  "quasi-occupational disease".


The clinical signs and the probably  non-allergic pathomechanism are comparable with those of lichen planus  induced by gold. In diseases due to occupational intoxication, we face an  individual disposition regarding the degree of clinical symptoms, which has  to underly any expert opinion on indemnity. 


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42.) [Lichen planus and lichen planus pigmentosus following gold therapy--case  reports and review of the literature]. 

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Z Hautkr 1986 Mar 1;61(5):315-9 

[Article in German] 


Ingber A, Weissmann-Katzenelson V, David M, Bialowons M, Feuerman EJ  We report on two typical cases of lichen planus and lichen pigmentosus  appearing after gold therapy. The characteristics of lichen planus induced  by drugs are emphasized, and the literature is reviewed. 


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43.) Lichen planus pigmentosus of the oral mucosa: a rare clinical variety. 

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Dermatologica 1981;162(1):61-3 


Laskaris GC, Papavasiliou SS, Bovopoulou OD, Nicolis GD 


In this paper, we describe a case of lichen planus of the mouth with  intense melanosis, in a middle-aged white male. Due to its unusual clinical  characteristics, we believe that this case represents a rare variant of  lichen planus of the oral mucosa. The histopathologic findings,  differential diagnosis and its possible connection with lichen planus  pigmentosus of the skin are discussed. 


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DATA-MÉDICOS/DERMAGIC-EXPRESS No (73) 29/09/99 DR. JOSÉ LAPENTA R. 

UPDATED 14 JUNE 2025

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