Nonmelanoma skin cancer in the setting of erosive pustular dermatosis of the scalp: A case series and comment on management implications.

BACKGROUND: Erosive pustular dermatosis of the scalp (EPDS) is an inflammatory cutaneous disorder typically affecting sun-damaged skin of mature individuals. Clinical features of EPDS include sterile pustules and chronic crusted erosions that can be hyperkeratotic and lead to scarring alopecia, atrophy, and telangiectasia. While the condition occurs on sun-damaged skin, a relationship with non-melanoma skin cancer (NMSC) has not been investigated. OBJECTIVES: Here we attempted to identify cases of NMSC developing in the setting of EPDS. METHODS: Retrospective review of EPDS cases in a dermatology practice. RESULTS: Six patients with mean (range) age 82 (65-92) years that developed NMSC in the setting of EPDS are reported. Five patients had skin phototype I or II associated with substantial solar elastosis. Four patients had history of NMSC. Four patients developed squamous cell carcinoma and two patients basal cell carcinoma on the scalp in the setting of EPDS. A morphologic change in an EPDS lesion, such as a crusted plaque becoming nodular and/or growing significantly within a relatively short period of time, prompted a biopsy that revealed NMSC. CONCLUSIONS: NMSC may develop in the setting of EPDS. Possible mechanisms underlying this association include the chronic inflammation associated with EPDS and ultraviolet light exposure. It is crucial to promptly obtain a biopsy in EPDS cases showing signs suspicious for NMSC. Further studies are required to confirm whether NMSC shows a higher prevalence in the setting of EPDS.

Aminolevulinic Acid Photodynamic Therapy in the Treatment of Erosive Pustular Dermatosis of the Scalp: A Case Series.

IMPORTANCE: Erosive pustular dermatosis of the scalp (EPDS) is an inflammatory skin condition that develops on sun-damaged skin in older individuals. Patients with EPDS present a therapeutic challenge because medical treatments and surgical modalities have met with limited success. Methyl aminolevulinate photodynamic therapy has been effective in 1 case but induced the disease in others. OBSERVATIONS: Eight patients with EPDS with mean (range) age 84 (67-93) years underwent gentle curettage of the hyperkeratotic lesions followed by aminolevulinic acid photodynamic therapy (PDT) 1 to 2 weeks later. Lesions resolved in 6 patients, whereas 2 patients had residual lesions at 6-week follow-up and underwent a second cycle of curettage and aminolevulinic acid PDT with resolution. One patient experienced a partial recurrence 5 months after the procedure and was successfully retreated with curettage + aminolevulinic acid PDT. No adverse effects were noted, and patients were satisfied with the treatment. CONCLUSIONS AND RELEVANCE: This series of patients demonstrated EPDS successfully treated with PDT. Lesions resolved in all patients with a protocol that included curettage followed by aminolevulinic acid PDT. Therefore, this protocol represents an efficacious modality for EPDS.

Primary cutaneous amyloidosis of the external ear: a clinicopathological and immunohistochemical study of 17 cases.

Primary cutaneous amyloidosis includes several forms of localized amyloidosis characterized by superficial amyloid deposits occurring at or near the dermal-epidermal junction in the absence of systemic involvement. Primary cutaneous amyloidosis of the auricular concha and external ear represents a rarely described variant. There have been 27 cases reported in the English language literature, and herein we report 17 additional cases. This article demonstrates that the amyloid observed in this context is generally positive for Congo red, crystal violet and thioflavin T. It also expresses cytokeratin 34ßE12 via immunohistochemistry. Our immunohistochemical results and review of the literature suggest that the amyloid in amyloidosis of the external ear is the result of basal keratinocyte degeneration and does not signify deposition from a systemic or generalized process.

De novo intraepidermal epithelioid melanocytic dysplasia: a review of 263 cases.

BACKGROUND: De novo intraepidermal epithelioid melanocytic dysplasia (DNIEMD) is a newly characterized lesion that is associated with a personal and/or family history of malignant melanoma (MM) and/or dysplastic nevi (DN). However, the biological significance is still uncertain and the persons predisposed to this lesion have not been adequately described. METHODS: Clinicopathologic data of 258 patients, from 263 biopsies diagnosed with DNIEMD, was obtained. A brief voluntary questionnaire was used to obtain demographic, risk factor and disease history. RESULTS: There is an 82% (n=263) predominance of women with DNIEMDs. For men and women, the distributions of these lesions occur on the lower extremities (71%), the upper extremities (24%) and trunk (5%). Thirty-one percent of the 258 patients responded to the questionnaires. 48% of the 60 respondents had green or blue eyes. 26% of 62 respondents had a history of non-melanoma skin cancer (NMSC). Combined data revealed that 68% of 134 patients had a history of DN. As well, 24% of 89 patients had personal histories of melanoma, while 24% of 72 patients had a family history of melanoma. CONCLUSION: Most of these DNIEMD lesions are found on the lower extremities of women and men, and they have an increased association with MM, DN and NMSC.

Incontinentia pigmenti: treatment of IP with topical tacrolimus.

Incontinentia pigmenti (IP), or Bloch-Sulzberger syndrome, is a rare X-linked dominant genodermatosis primarily affecting females. Although IP affects many organ systems, the hallmark feature of this disease is its characteristic cutaneous eruption along the lines of Blaschko that evolves through four distinct stages: inflammatory/vesiculobullous, verrucous, hyperpigmented and hypopigmented/ atrophic. We describe a case of IP in its vesicular stage that completely resolved with topical Protopic (tacrolimus) 0.1% ointment. The treatment successfully halted the progression of disease through its subsequent disfiguring stages.

Clear cell fibrous papule with NKI/C3 expression: clinical and histologic features in six cases.

Fibrous papule of the nose is a common benign lesion of dermal fibroblast lineage. Two unusual variants have been described, namely, fibrous papule with granular cells and fibrous papule with clear fibrocytes. We report a second case series (six cases) of clear cell fibrous papule to add to the first series of 9 cases. Clinical and histologic features in our cases are similar to those in the first series. All of our specimens were dome-shaped, 2- to 5-mm skin-colored to slightly erythematous papules on the faces of three male and three female adults ranging from 18 to 48 years of age. All but one lesion were on the nose. Clinical differential diagnoses included fibrous papule, verruca, basal cell carcinoma, and a variety of other neoplasms. Histologically, dermal aggregates of clear cells with finely granular to vacuolated cytoplasm, and centrally located nuclei, were found. Most specimens also contained ectatic capillaries, and all showed evidence of irritation or trauma. Periodic acid-Schiff stain was negative in all specimens to which it was applied (5/6). Neural, melanocytic, and epithelial origins were eliminated by negative staining with S-100, Mart-1, cytokeratins, epithelial membrane antigen, and carcinoembryonic antigen, performed on some of the specimens. A mesenchymal nature was confirmed in one specimen staining strongly positive for vimentin. Five of six cases stained positively for CD68, and all five cases studied were strongly and diffusely positive for NKI/C3. Factor XIIIa stain highlighted scattered dendritic cells within the lesion but was otherwise negative in all six cases studied. Recognition of this variant of fibrous papule is important to distinguish this benign lesion from other clear cell neoplasms.

Local melanoma recurrence: a clarification of terminology.

BACKGROUND: The current medical literature contains multiple different meanings for the term "local melanoma recurrence." Confusion regarding locally persistent and locally metastatic disease makes interpretation and analysis of previously published reports difficult if not impossible. OBJECTIVE: The objective was to present a more precise definition of local melanoma recurrence. METHODS: A case is reported and the literature is reviewed. CONCLUSION: Owing to the myriad of different definitions that exist in the medical literature, the term "local melanoma recurrence" is ambiguous and at times misleading. Melanoma that recurs locally from persistence of tumor at the resection margins has a vastly different prognosis than recurrence developing from local (satellite) metastases adjacent to the surgical resection site. We propose the use of the terms "persistent melanoma" and "local metastasis" as more precise and predictive of a patient's prognosis.

Specific dermatoses of pregnancy: an evidence-based systematic review.

OBJECTIVE: We conducted an evidence-based systematic analysis of the literature on specific dermatoses of pregnancy. STUDY DESIGN: The bibliographic databases MEDLINE and EMBASE were screened for studies and reports in all languages about herpes gestationis, pruritic urticarial papules and plaques of pregnancy, pruritic folliculitis of pregnancy, and prurigo of pregnancy from January 1962 to January 2002. As main index terms, including analogs and derivatives, we used the names of specific dermatoses of pregnancy. Intrahepatic cholestasis of pregnancy, not a primary dermatosis, was included herein because this disorder is associated with pregnancy and its secondary skin manifestations must be differentiated from specific dermatoses of pregnancy. Other sources were abstract books of symposia and congresses, theses, textbooks, monographs, reviews, editorials, letters to the editor, free or rapid communications, and the reference lists from all the articles that were retrieved. All articles selected for inclusion in this review were evaluated critically with regard to their impact factor and evidence-based contribution to this field, as measured by their citation index and impact factor of the journal in which they were published. Approximately 39% of articles met the selection criteria. RESULTS: The clinical features and prognosis of the specific dermatoses of pregnancy have been delineated through a number of retrospective and cohort studies. The molecular biologic and immunogenetic properties of herpes gestationis, pruritic urticarial papules and plaques of pregnancy, and intrahepatic cholestasis of pregnancy have been further clarified. A meta-analysis in this review reveals a higher prevalence of multiple gestation pregnancy (11.7%) among patients with pruritic urticarial papules and plaques of pregnancy. Several investigations have unraveled the fetal complications in intrahepatic cholestasis of pregnancy and herpes gestationis. New treatment modalities in intrahepatic cholestasis of pregnancy (cholestyramine, ursodeoxycholic acid) and herpes gestationis (cyclosporin, intravenous immunoglobulin, and tetracyclines postpartum) have shown promise and warrant further evaluation. CONCLUSION: During the past few decades, a significant amount of new data has provided new insights into the classification, pathogenesis, treatment, prognosis, and fetal risks that are associated with the specific dermatoses of pregnancy.

Lentigo maligna with spread onto oral mucosa.

BACKGROUND: Lentigo maligna (LM) is a form of melanoma in situ most often seen in white patients on sun-exposed areas, primarily the head and neck. Spread of LM onto the conjunctiva has been reported. There have been no reports of LM extending onto oral mucosa. OBSERVATIONS: We report 4 cases of LM in white women with contiguous spread from perioral areas to oral mucosa. The locations of the primary lesions were the vermilion of the lip, vermilion and perioral skin, cheek, and cutaneous aspect of the lip. Three cases showed focal histopathologic evidence of invasion during the course of the disease. The lesions ran a prolonged course characterized by repeated recurrences after surgery. Three of the cases required a complicated reconstruction after surgical excision. Mohs surgery with rush permanent (paraffin-embedded) sections resulted in a long remission in 2 cases, while in 1 patient, treatment with carbon dioxide laser was unsuccessful. CONCLUSIONS: In a perioral distribution, LM can spread onto oral mucosa. This clinical presentation may cause significant long-term morbidity, as indicated by a high recurrence rate and/or progression to invasive melanoma. The oral mucosa should be examined in patients with atypical pigmented perioral lesions.

Linear IgA bullous dermatosis occurring after carbamazepine.

Linear IgA bullous dermatosis (LABD) is an immunobullous disorder in which IgA antibodies are deposited within the basement membrane zone. The cause is unknown, but the eruption may occur in association with certain medications. We report a patient who experienced LABD shortly after starting carbamazepine therapy.

Intradermal melanocytic nevus with prominent schwannian differentiation.

Features of peripheral nerve sheath differentiation such as neuroid cords, nerve corpuscles, fascicle-like structures, and, exceptionally, palisading have been reported in melanocytic nevi. We report an intradermal melanocytic nevus with prominent Verocay-like bodies. The upper portion of the neoplasm was composed of typical round intradermal nevus cells, many of which were pigmented. Within the deeper portion, there was a nonpigmented spindle cell proliferation with prominent Verocay bodies, simulating a neurilemmoma. Typical nevus nests merged with neurilemmoma-like areas. The entire lesion stained positively for S-100 and Mart-1 proteins and negatively for HMB-45 stain. Diffuse Mart-1 positivity excluded a collision of a melanocytic lesion with a neurilemmoma. The histopathologic features of this nevus further support a close relation between nevus cells and Schwann cells.