Lichen sclerosus and immunobullous disease.

We report 3 patients with long-standing lichen sclerosus who subsequently developed new onset erosive disease in affected sites. Repeated biopsies were performed which, although not diagnostic, showed some features of bullous pemphigoid for 1 patient and nonspecific findings for the 2 others. Direct immunofluorescence showed the characteristic findings of bullous pemphigoid in the first and pemphigus vulgaris in the others. All 3 patients were treated with immunosuppressive agents, and their condition improved dramatically.

Rapid resolution of primary vulval adult Langerhans cell histiocytosis with very potent topical corticosteroids.

Langerhans cell histiocytosis is a rare idiopathic disorder with protean clinical presentations. Primary unifocal single-system disease of the vulva is even less common. We report a 62-year-old female patient presenting with an 18-month history of pruritus and burning of the vulva. Clinical examination of the vulva showed a tender nodule of the right labium minus. Histology confirmed Langerhans cell histiocytosis. Systemic involvement was excluded. Within 1 month the use of clobetasol propionate ointment led to resolution of both the patient's symptoms and the clinical appearance of the affected right labium minus. This resolution was maintained 12 months later.

Superficial granulomatous pyoderma of the vulva in a patient receiving maintenance rituximab (MabThera) for lymphoma.

BACKGROUND: Vulval ulceration can be caused by a wide variety of etiological factors including bacterial and viral infections, granulomatous disorders, and malignancy. Superficial granulomatous pyoderma (SGP) is a variant of pyoderma gangrenosum. It is characterized by localized ulcerative lesions that may be precipitated by surgery. We report a case of vulval SPG in an immunocompromised patient. CASE: A 51-year-old woman presented with a 6-week history of severe vulval pain, bleeding, and rapidly progressing ulceration. She had a previous history of relapsed follicular non-Hodgkin lymphoma and was currently receiving regular MabThera (Welwyn Garden City, Hertfordshire, UK). Examination revealed deep ulceration involving the entire vulva and extending into the vagina with areas of necrosis. Histological examination showed ulceration with sparse granulomas and eosinophils. The clinical and histological findings confirmed a diagnosis of SGP. CONCLUSIONS: Vulval ulceration in an immunocompromised patient has a broad differential diagnosis. The possibility of a granulomatous condition such as SGP must always be considered.

Cutaneous manifestations of internal malignancy: diagnosis and management.

An association between systemic malignancy and cutaneous manifestations has long been recognized. The cutaneous features that can occur are numerous and heterogeneous, and many different etiologic mechanisms are represented - from direct tumor invasion of skin or distant metastases to a wide variety of inflammatory dermatoses that may occur as paraneoplastic phenomena. In addition, there are a number of inherited syndromes that carry an increased risk of cutaneous as well as internal malignancies. While some of these inherited syndromes and paraneoplastic phenomena are exceedingly rare, all clinicians will be aware of the common cutaneous manifestations of advanced malignant disease such as generalized xerosis and pruritus. This review classifies these wide-ranging cutaneous manifestations of internal malignancy into five basic groups and provides practical advice regarding diagnosis and screening of patients who initially present with a cutaneous complaint. Also included is up-to-date information on two rapidly expanding and exciting areas of research that are likely to have far-reaching clinical implications: (i) clarification of underlying humoral mechanisms, for example, in the malignant carcinoid syndrome; and (ii) identification of an increasing number of specific genetic defects that confer a susceptibility to malignancy.Increased clinician awareness regarding the associations between these lesions and internal malignancy or inherited syndromes will facilitate screening and early diagnosis.

Neonatal giant congenital nevi with proliferative nodules: a clinicopathologic study and literature review of neonatal melanoma.

BACKGROUND: Review of the literature reveals that congenital malignant melanoma is an exceptionally rare occurrence and has a generally poor prognosis when it does occur. However, benign proliferative melanocytic lesions are known to occur within giant congenital nevi (GCN). This entity is not well recognized and can be confused clinically and histologically with malignant change. OBSERVATIONS: We report 2 cases of GCN in neonates demonstrating benign proliferating nodules present at birth. An initial diagnosis of malignant melanoma was assumed in both cases. Careful histologic analysis, however, revealed these lesions to be benign, as did long-term follow-up of 3.5 years, with both patients remaining well with no evidence of melanoma. Review of the literature suggests that there are 2 clinical patterns of these benign nodules arising within GCNs: small (<1 cm) and large (>1 cm) dermal nodules with varying histologic patterns that we have attempted to categorize. CONCLUSIONS: Our cases illustrate the difficulty in accurate diagnosis of melanocytic lesions in the neonate. We recommend caution in making a diagnosis of malignant melanoma and highlight the possibility that benign lesions can be mistaken for melanoma in this age group. We encourage the acquisition of fixed histologic specimens for accurate diagnosis of melanocytic lesions.