[Sclerodermatous changes in porphyria cutanea tarda: six cases]. / Lésions sclérodermiformes dans la porphyrie cutanée tardive : six observations.

BACKGROUND: The clinical features of porphyria cutanea tarda (PCT) are usually distinctive and include blistering on sun-exposed areas, fragile skin, hypertrichosis and hyperpigmentation. Sclerodermatous changes are much less common, and may either reveal PCT or else appear later. We carried out a retrospective study of the files of six female patients presenting such lesions. PATIENTS AND METHODS: Six women (age: 45 to 72 years) were referred for sclerodermatous lesions on sun-exposed areas of the upper body. In four patients, these lesions revealed PCT and in the remaining two patients they were indicative of previously treated but relapsing PCT. Four had sclerodermatous skin changes mimicking morphea of the neck and neckline, the top of the back and the face, while one presented more diffuse facial and cervical sclerosis. Associated alopecia was seen in three patients. The last patient presented isolated sclerodermiform alopecia. Associated malar hypertrichosis was seen in five cases and facial hyperpigmentation was noted in three cases. Four exhibited no blisters, cutaneous fragility, milia or photosensitivity. Histological findings were consistent with morphea or scleroderma in all cases. All patients presented abnormal liver tests: cirrhosis was present in four cases (primitive biliary cirrhosis, alcoholic cirrhosis and hepatitis C) and fatty liver in two cases. In four cases, there was excessive alcohol intake. Uroporphyrin levels were above the normal range in all cases. Local corticosteroid therapy associated with phlebotomy and/or low-dose hydroxychloroquine resulted in complete normalisation of porphyrin levels in four patients, with complete resolution of the cutaneous lesions in two patients and partial improvement in the other two. DISCUSSION: Sclerodermatous changes are uncommon in PCT. They are not always late and secondary to the process of healing of blisters but can in fact constitute the first cutaneous symptom of the disease while revealing the underlying liver disease. Even in the absence of blisters, photosensitivity or cutaneous fragility, a diagnosis of PCT must be suspected in a setting of sclerodermatous changes distributed on the neck and face, or the neckline, or scarring alopecia, if associated with abnormal liver tests. Skin biopsy to confirm the diagnosis of scleroderma may delay the diagnosis, which is in fact based on porphyrin level. Normalization of the latter parameter under treatment allows regression of lesions.

[Disseminated perinaevic Meyerson phenomenon revealing melanoma]. / Phénomène de Meyerson périnævique disséminé révélateur d'un mélanome.

BACKGROUND: Meyerson phenomenon (MP) consists of an eczematous reaction occurring around a pre-existing dermatologic lesion that is usually melanocytic and generally benign, and which is known as a Meyerson naevus. We report a case of multiple Meyerson naevi revealing melanoma, which itself was surrounded by a halo of eczema. PATIENTS AND METHODS: A 55-year-old man of phototype III with atopic eczema presented for pruritic eczema present for a fortnight, found solely on and around the naevi on his trunk and at roots of his limbs. One of the melanocytic lesions affected by these Meyerson phenomena was clinically atypical and had been active for several years. Excision confirmed the diagnosis of level II extensive superficial melanoma measuring 0.75 mm in thickness and associated with lesional and perilesional eczematous remodelling. After surgery involving a 1-cm excision margin and local corticosteroid therapy of the eczema, the Meyerson phenomenon subsided with complete remission of the melanoma at 1 year. DISCUSSION: Meyerson phenomenon can affect one or more naevi at the same time; it is generally transient, may recur on occasion, and has a favourable outcome either spontaneously or with corticosteroid treatment. When not removed for histological verification, the melanocytic lesion regains its initial appearance following resolution of the phenomenon. MP differs from Sutton phenomenon (SP), which is a perinaevic vitiligo reaction leading to complete or partial regression of the melanocytic lesion, which may be either benign or malignant. CONCLUSION: This case of Meyerson phenomenon revealing melanoma shows that the melanocytic lesions targeted by MP are not necessarily benign.

[Discoid lupus erythematosus involving the eyelids: 4 cases]. / Blépharites du lupus érythémateux chronique. Quatre cas.

BACKGROUND: Discoid lupus erythematosus is a chronic skin disease frequently involving the face, scalp and ears. Palpebral lesions are rare. We report 4 cases of this uncommon localization. In one case, palpebral lesions were the sole manifestation of the discoid lupus erythematosus. CASE REPORTS: Four patients aged between 26 and 45 Years had lesions of the lower eyelid as erythematous, infiltrated and scaly plaques. One patient, without diagnosis despite 2 skin biopsies of lesion, presented with a 2-Year history of blepharitis in the absence of any other cutaneous abnormality. In one case, chronic blepharitis has been present for 4 Years and associated with inflammatory arthralgia. Alopecia occurred 4 Years after the onset of blepharitis and led to the diagnosis of discoid lupus erythematosus. In 2 cases, blepharitis was associated with typical cutaneous lesions of discoid lupus erythematosus. Antimalaria drugs were very effective in 3 cases. In one patient the antimalaria drug failed, but thalidomide was effective. DISCUSSION: The location of lesions on the eyelids in the absence of any other cutaneous abnormality is rare and can easily lead to the misdiagnosis of discoid lupus erythematosus. Most Authors mention a predilection of the lesions to the inferior portion of the eyelid, more specifically to the external third. The involvement of the eyelids with permanent scarring and severe eye impairment is explained by the long duration of the disease without diagnostic and appropriate treatment. Clinical examination is highly consistent with discoid lupus erythematosus: the lesions typically present as well-circumscribed, erythematous plaques with telangiectasia and scales and atrophy in long-standing disease. Antimalaria drugs are remarkably effective.

[Photochemotherapy for pityriasis lichenoides: 3 cases]. / RePUVAthérapie dans le pityriasis lichénoïde: 3 cas.

BACKGROUND: Pityriasis lichenoides is a rare cutaneous disorder of unknown origin that mainly affects children and young people. The disease consists of two variants: pityriasis lichenoides et varioliformis acuta, characterized by papular lesions evolving into necrosis and pityriasis lichenoides chronica characterized by scaly papules that may last from several months to several years. Various types of therapy have been proposed for pityriasis lichenoides, to our knockledge, photochemotherapy has never been used. We report the first 3 cases of pityriasis lichenoides treated by photochemotherapy. CASE REPORTS: Three patients, 6, 15 and 18 years old, presented respectively with a 1 month, 2 years and 3 months history of numerous, papular and necrotic lesions or squally papular lesions. Clinical appearance and histopathologic examination of biopsy were consistent with pityriasis lichenoides et varioliformis acuta (patients 1 and 3) and with pityriasis lichenoides chronica (patient 2). They had received different treatments without significant effect: topical corticosteroids, antibiotic, UVB therapy and dapsone. Acitretin associated with PUVA were dramatically effective within few weeks. DISCUSSION: Pityriasis lichenoides can be a severe scarring disease with significant social, psychological and physical consequences. In our opinion, photochemotherapy, which has never been used in pityriasis lichenoides, is effective. Therapeutic modalities have to be define.

[Localized bullous pemphigoid induced by thermal burn]. / Pemphigoïde localisée autour d'une brûlure thermique.

BACKGROUND: Induced bullous pemphigoid is known to be triggered by drugs and some physical agents. Six cases of bullous pemphigoid induced by thermal burns have been reported since 1991. CASE REPORT: We observed a bullous eruption around thermal burns of the left leg in a 89 year-old woman. Bullous pemphigoid was diagnosed by histological and immunohistochemical findings with a sub-epidermal blister and linear IgG and C3 deposition at the dermal-epidermal junction. Indirect immunofluorescence was negative. The eruption rapidly resolved with topical steroids. DISCUSSION: We describe a bullous pemphigoid induced by a thermal burn. This case is original because the eruption was localized only around the site of the burn and healed with topical steroids. In the literature, there are 6 other cases reported but only one localized. We discuss the hypothesis of asymptomatic bullous pemphigoid exacerbated by presentation of bullous pemphigoid antigen by thermal burns or self immunization against antigens altered by burns.

Lipodystrophy associated with protease inhibitors.

Lipodystrophies, characterized by reduction of subcutaneous fat over part or all of the body surface, are uncommon. Their causes are unknown. Recently, lipodystrophy has been reported in human immunodeficiency virus (HIV)-infected patients taking protease inhibitors, which have been recommended since 1996 as standard therapy for HIV disease in combination with nucleoside analogues. In these cases, lipodystrophy consists of an association of peripheral lipoatrophy with central adiposity. We report four HIV-infected men on protease inhibitors who developed a disfiguring lipodystrophy. In three of them, the protease inhibitor was administered for a mean duration of 21.5 months (range 19-23) with good immunological and virological responses. Patient 4 had been treated for 2 years with successive combinations of protease inhibitors with nucleoside analogues without success. The four patients progressively developed an increase in abdominal girth associated with fat wasting of the face and legs. Two of them had recurrent paronychia of the great toes. Triglyceride levels were moderately increased in all patients, and one had a slightly increased cholesterol level. One patient had elevated glucose and insulin plasma levels during a glucose tolerance test. In two patients, a deep biopsy taken from the thigh showed thinning of the subcutaneous fat without other morphological changes. Computed tomographic scans of the face and abdomen confirmed the loss of almost all subcutaneous fat of the cheek and temporal regions, and abdominal perivisceral fat accumulation. For patients 1-3, the protease inhibitor was replaced by a non-nucleoside reverse transcriptase inhibitor. Nine months later, dysmorphic changes had not regressed, but lipid abnormalities had returned to normal and the paronychia had disappeared.