Eosinophilic annular erythema: A subset of Wells' syndrome or a distinct entity?

A 52-year-old woman with a 6-year history of a persistent non-pruritic cutaneous annular eruption, forming polycyclic and arcuate plaques that commenced as erythematous papules and nodules, is presented. Lethargy and arthralgia were associated symptoms. We have followed this patient for the last 3 years, and during this period she has continued to have a florid annular eruption of unknown cause. Laboratory tests, including an eosinophil count, examination of stool samples for parasites, and a computed tomography scan of the chest, abdomen and pelvis, failed to detect any abnormalities. Skin biopsies demonstrated a superficial to deep cellular infiltrate consisting of numerous eosinophils, with lymphocytes and isolated neutrophils. Eosinophilic dust, flame figures and granulomatous inflammation were not seen. In addition, strands of mucin were present through the dermis, and prominent basal vacuolar change was evident at the dermoepidermal junction; these features may represent new findings that help define a distinct form of eosinophilic annular erythema.     

Necrolytic acral erythema: a variant of necrolytic migratory erythema or a distinct entity?

BACKGROUND: Hepatitis C is a major health problem in Egypt. Necrolytic acral erythema (NAE) is a recently described necrolytic erythema that has a distinctive acral distribution and a uniform association with hepatitis C. Some authors believe that NAE is a distinct entity and others consider it as a variant of necrolytic migratory erythema (NME). METHODS: Five patients with clinical features consistent with NAE were included in this study. The patients were subjected to skin biopsy examination, CT scan of the pancreas and a liver biopsy. Liver function tests, serum glucagon, glucose, amino acids and zinc were measured. All patients were tested for hepatitis C by enzyme-linked immunosorbent assay (ELISA) and by polymerase chain reaction (PCR). RESULTS: Three patients presented with early (acute) lesions and two patients with chronic lesions. The distribution of the lesions was almost exclusively on the dorsae of the feet. Histopathological findings were similar to those of other necrolytic erythemas. Hepatitis C virus was uniformly detected in all patients. Serum glucagon was high in two patients, serum glucose was high in four patients, serum amino acids were low in three cases and serum zinc and albumin were low in two cases. Little or no improvement was reported after oral amino acid supplementation, while the response to oral zinc sulfate was moderate to good. CONCLUSION: Necrolytic acral erythema is closely associated with hepatitis C infection. Many findings indicate that NAE seems to be a variant of NME rather than a distinct entity. Hence, an alternative proposed term could be acral NME.     

Erythematous dermatosis with fibroplasia: a new entity?

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A case of acantholytic dermatosis and leukemia cutis: cause or effect?

Leukemia cutis is capable of presenting in a variety of clinical and histologic guises. We describe a 75-year-old man with a recent diagnosis of M0 acute myelogenous leukemia, who presented with multiple pruritic erythematous papules on his chest and back. Microscopically, the epidermis showed acrosyringeal-based acantholysis consistent with transient acantholytic dermatosis (TAD), associated with exocytosis of atypical hematolymphoid cells. In addition, the dermis showed a contiguous atypical hematolymphoid proliferation consistent with conventional leukemia cutis. To our knowledge, this is the first such case combining features of TAD with leukemia cutis. It remains to be determined whether the acantholysis occurred secondary to the leukemia cutis, was initiated by the migration of leukemic cells, or if the association is merely serendipitous.     

Predominant telangiectatic erythema in linear atrophoderma of Moulin: novel variant or separate entity?

Linear atrophoderma of Moulin is a distinctive disease originally described in 1992 and characterized by acquired, mildly atrophic, non-sclerotic, slightly hyperpigmented lesions following the lines of Blaschko. Here, we describe a 15-year-old girl with a 13-year history and a 29-year-old male with a 6-year history of prominent linear telangiectatic erythema and mild atrophoderma following the lines of Blaschko that involved the right leg and hip, and both legs, the trunk and both arms, respectively. As pronounced telangiectatic erythema within lesions of atrophoderma of Moulin has not hitherto been described, we propose that the disease in our patients represents a novel variant of linear atrophoderma of Moulin. Due to considerable overlap, we do not favour the notion that our cases constitute an entity entirely separate from linear atrophoderma of Moulin.     

Nonmutilating palmoplantar and periorificial kertoderma: a variant of Olmsted syndrome or a distinct entity?

Background Olmsted syndrome is a rare keratinization disorder characterized by mutilating palmoplantar and periorificial keratoderma as the two major diagnostic features. Some authors believe that atypical cases without this standard combination may not really belong to Olmsted syndrome. Herein, we describe two familial cases with congenital nonmutilating palmoplantar and periorificial keratoderma, and discuss their similarities and differences with Olmsted syndrome. Patients The study included two sisters who presented with focal and punctate nonmutilating palmoplantar keratoderma (PPK), periorificial hyperkeratotic plaques, and widely distributed keratotic lesions. Fragile denuded areas of the skin were found in sites exposed to trauma. Fingernails showed a characteristic form of leukonychia. Results Histopathology of plantar keratoderma showed psoriasiform hyperplasia with marked compact hyperkeratosis, while vicinity of denuded skin revealed thin parakeratotic zone and dissolution of the granular cell layer. Immunohistochemistry demonstrated suprabasal staining pattern for acidic keratin (AE1) and uniform positivity, starting four to six layers above the basal layer, for cytokeratin 10. Electron microscopy showed defective keratinization. Cytogenetic studies revealed normal karyotype and no chromosomal breakage. Conclusion Our cases share Olmsted syndrome in the early onset, and the presence of symmetrical PPK, periorificial keratoderma and keratotic lesions. However, the striking nonmutilating nature of PPK and the presence of unique features in our patients suggest a newly described keratinization disorder.     

Sclerodermiform linear lupus erythematosus: A distinct entity or coexistence of two autoimmune diseases?

The coexistence of features of chronic cutaneous lupus erythematosus and scleroderma in the same skin lesions is very infrequent and has only been reported in 3 patients. This exceedingly rare condition has been named "sclerodermiform linear lupus erythematosus." We describe a new case of this dermatosis. Although the cause remains obscure, possible explanations include mosaicism following Blaschko's lines or the transfer of microchimerisms that mount a chronic graft-versus-host-like reaction. We suggest that these features may be a distinct clinicopathologic disorder characterized by an initial lichenoid reaction followed by the production of excessive, abnormal connective tissue.     

Hypopigmented keratosis: is it a hyperkeratotic variant of idiopathic guttate hypomelanosis?

We have occasionally seen patients with acquired well‐demarcated, scattered hypopigmented papules. In this study, we investigated the clinical and histopathological characteristics of such lesions. Biopsies were taken from the lesional and perilesional normal skin from 10 of 13 patients, which were compared with 10 idiopathic guttate hypomelanosis (IGH) samples. The lesions were scattered, well‐circumscribed, flat‐topped, hypopigmented papules. There was no age or gender predilection. Marked hyperkeratosis was present, with clear‐cut margins distinguishable from the adjacent normal epidermis. The melanin content was decreased in the lesional epidermis, which was associated with a decrease in expression of melanogenesis‐associated markers such as tyrosinase and NKI/beteb (marker of gp100) and reduction in the number of melanocytes. These histological findings were similar to those of IGH except for the additional finding of a thicker stratum corneum in this case seem to represent a ‘hyperkeratotic’ variant of IGH.