Lichen amyloidosis of the scalp and forehead.

Lichen amyloidosis is a subtype of primary localized cutaneous amyloidosis (PLCA), which presents as discrete, firm, closely-set 1-3mm, dome-shapedbrown papules commonly involving the anterior aspect of shins and extensor surfaces of forearms. We present a case of an otherwise healthy man in his 30s with solitary facial involvement of lichen amyloidosis, which is very uncommon.

Tick Bite Alopecia: A Report and Review.

Tick bites can cause a number of local inflammatory reactions, which are often difficult to differentiate from those induced by other arthropod bites or stings. These include erythematous nodular or pustular lesions, erosive plaques, annular lesions of erythema chronicum migrans, and both scarring and nonscarring inflammatory alopecia. We report a case of nonscarring alopecia in a 21-year-old male who reported a recent history of tick bite to the scalp. The biopsy demonstrated a dense pseudolymphomatous inflammatory infiltrate with numerous eosinophils associated with hair follicle miniaturization and an elevated catagen-telogen count. Signs of external rubbing, including lichen simplex chronicus and the "hamburger sign", were also visualized and are indicative of the associated pruritus. To the authors' knowledge, this is the fifth report of nonscarring tick bite alopecia in the literature and the first in an adult patient. This text will review the classic clinical presentation, histologic findings, and proposed mechanism of tick bite alopecia.

Lentigo Maligna Melanoma With Local and Distant Blue Nevus-like Metastases.

Melanoma or melanoma metastases can rarely mimic blue nevi clinically and/or histologically, presenting a diagnostic pitfall for both the clinician and the dermatopathologist. We report a case of an invasive lentigo maligna melanoma with subsequent development of multiple, cutaneous blue nevus-like localized metastases followed by a distant metastasis, heralding widespread systemic metastases.

A case of inflammatory nonscarring alopecia associated with the tyrosine kinase inhibitor nilotinib.

IMPORTANCE: Nilotinib, a recently approved multitargeted tyrosine kinase inhibitor targeting the BCR-Abl translocation involved in chronic myelogenous leukemia, reportedly produces alopecia according to the package insert, but clinical and histologic descriptions of the alopecia are lacking. OBSERVATIONS: A 33-year-old woman with chronic myelogenous leukemia developed widespread alopecia involving scalp and body hair within weeks after starting nilotinib therapy. Biopsies revealed perifollicular lymphocytic inflammation and evidence of follicular injury but normal hair density, consistent with a nonscarring alopecia. CONCLUSIONS AND RELEVANCE: Nilotinib therapy may induce perifollicular inflammation and widespread persistent alopecia. We present the first clinical and histologic description of this potential adverse effect. Further investigation into the underlying mechanism of this adverse effect may produce insights into the hair growth cycle as well as potential therapeutic targets.

Eczematous nevus sebaceus: a report of three cases.

Meyerson's phenomenon is a well-documented inflammatory reaction described in a variety of cutaneous lesions, including the original description in nevocellular nevi (1). Such an inflammatory reaction was subsequently described in melanocytic and a sundry of nonmelanocytic lesions alike, including vascular malformations (2-11). We present three cases of infants with nevus sebaceus on the scalp, which were obscured by an eczematous, eosinophilic reaction reminiscent of that first described by Meyerson. Two of the patients had concomitant atopic dermatitis, but one had no association. Recognition of this secondary feature is important in establishing the correct diagnosis.

Histiocytic erythema multiforme.

Erythema multiforme is histologically characterized by liquefactive degeneration along the dermal-epidermal junction, necrotic keratinocytes and a lymphocytic infiltrate. We report a 10-year-old boy with recurrent erythema multiforme major of undetermined etiology with unusual histologic findings. A skin biopsy taken at day 2 of his eruption revealed histologic features otherwise characteristic of erythema multiforme, but mediated instead by a CD68-positive infiltrate, resembling cutaneous Kikuchi's disease. To the best of our knowledge this is the first reported case of 'histiocytic' erythema multiforme.

Interstitial granulomatous drug reaction to anakinra.

Interstitial granulomatous drug reactions are an uncommon entity presenting as asymptomatic, annular, erythematous to violaceous plaques. The incidence of such reactions has been increasing with the use of biologic agents. We report, to the best of our knowledge, the first such reaction to the interleukin (IL)-1 inhibitor anakinra. Our patient presented with pink dermal plaques and nodules in the periaxillary region which resolved with discontinuation of anakinra and recurred upon restarting anakinra. Biopsy revealed a diffuse dermal infiltrate of lymphocytes and histiocytes with interspersed neutrophils and eosinophils. Fragmentation and degeneration of collagen and elastic fibers was also present. Withdrawal of anakinra led to complete resolution of the lesions. Interstitial granulomatous drug reactions are increasing in frequency and we add anakinra to the list of causative agents.

Reactive angioendotheliomatosis in association with a well-differentiated angiosarcoma.

A 55-year-old white female with a complex medical history including mixed connective tissue disease and peripheral vascular disease developed a group of red-purple papules on her proximal medial thigh that was followed, five months later, by the development of a large violaceous patch. She reported a history of radiation to this site (for melanoma) during her childhood. She was admitted to the hospital with a presumptive diagnosis of cellulitis, but failed to respond to antibiotics. A biopsy was performed and demonstrated a well-differentiated angiosarcoma arising in conjunction with reactive angioendotheliomatosis. Excision of the lesion was performed, and fifteen months of follow-up have shown no recurrence or metastasis.

Lymphoepithelioma-like carcinoma of the skin with spindle cell differentiation.

BACKGROUND: Primary cutaneous LELC is a cutaneous neoplasm with histopathologic features identical to those seen in the undifferentiated subtype of nasopharyngeal carcinoma. It is extremely rare, with only approximately 30 cases reported in the literature. METHODS: We report a case of primary cutaneous LELC arising on the forehead of a 72 year-old male in which a proportion of the neoplastic cells demonstrated distinctive spindle cell morphology. RESULTS: Microscopic examination showed a dense lymphoplasmacytic infiltrate admixed with large spindle-shaped cells with vesicular nuclei, prominent nucleoli, and frequent mitotic figures. These cells were negative for an extensive panel of immunohistochemical markers and positive only for broad-spectrum cytokeratins and epithelial membrane antigen. There was no connection between the tumor and the epidermis and no epidermal dysplasia. In situ hybridization for Epstein-Barr virus was negative. CONCLUSIONS: The spindle cell differentiation in this case is unusual and suggests that in some cases the differential diagnosis of cutaneous spindle cell neoplasms might include primary cutaneous LELC.

Death receptor apoptosis signaling mediated by FADD in CD30-positive lymphoproliferative disorders involving the skin.

BACKGROUND: The CD30-positive lymphoproliferative disorders lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (C-ALCL), and systemic anaplastic large cell lymphoma (S-ALCL) are lesions that overlap clinically, histopathologically, and immunophenotypically. Their biologic behaviors, however, vary considerably. In particular, lesions of LyP regress spontaneously while those of S-ALCL persist and often progress. Apoptosis has been suggested as the mechanism by which the lesions of LyP regress, but the underlying signaling pathways remain unclear. In this study, we used newly developed activation state-specific antibodies to demonstrate apoptosis signaling through the death receptor-mediated pathway regulated by FADD and caspase 3. METHODS: Dual immunohistochemistry for CD30 and activated forms of FADD and caspase 3 was performed on cutaneous biopsy specimens from 27 patients with CD30-positive lymphoproliferative disorders involving the skin. The patients included 18 with primary cutaneous CD30-positive LPDs (15 with LyP and 3 with C-ALCL) and 9 with S-ALCL. RESULTS: The proportion of CD30-positive cells expressing activated FADD was significantly different between primary cutaneous CD30-positive lymphoproliferative disorders and S-ALCL (36.4% vs. 14.5%, P = 0.0083). Expression of cleaved caspase 3 was also significantly different between primary cutaneous lesions and S-ALCL (9.2% vs. 1.9%, P = 0.048). CONCLUSIONS: Although a larger number of cases should be studied to validate these results, these data provide evidence that differences in signaling through the death-receptor apoptosis pathway mediated by FADD may be responsible for the varying biologic behaviors of CD30-positive lymphoproliferative disorders involving the skin.

Neutrophilic eccrine hidradenitis: a case report of an unusual annular presentation.

Neutrophilic eccrine hidradenitis (NEH) is an acute, self-limited, inflammatory neutrophilic dermatosis. It is most commonly described in patients with acute myelogenous leukemia (AML) following chemotherapy. NEH generally presents as erythematous papules and plaques on the head, trunk, and extremities. Histologically, NEH is characterized by a neutrophilic infiltrate surrounding eccrine ducts and coils with occasional necrosis. We describe a case of NEH with an unusual presentation of annular plaques. A search of the literature revealed only one other case report of NEH presenting as an annular eruption.

Sebaceous carcinoma, basal cell carcinoma, trichoadenoma, trichoblastoma, and syringocystadenoma papilliferum arising within a nevus sebaceus.

BACKGROUND: Nevus sebaceus has a well-documented potential to develop a wide variety of neoplasms of both epidermal and adnexal origins. It is highly unusual for more than three tumors to arise simultaneously within a single nevus sebaceus. Sebaceous carcinoma arising within a nevus sebaceus is a rare occurrence. OBJECTIVE: The objective was to report the case of a patient with a nevus sebaceus that simultaneously developed five distinct neoplasms of epidermal and various adnexal origins and to report the fourth case of sebaceous carcinoma arising within a nevus sebaceus. METHODS: A 45-year-old woman presented with a nevus sebaceus that contained five separate neoplasms, including sebaceous carcinoma, basal cell carcinoma, trichoadenoma, trichoblastoma, and syringocystadenoma papilliferum. RESULTS: Complete excision of the nevus sebaceus and the five tumors was performed. Systemic work-up showed no evidence of metastatic disease or association with Muir-Torre syndrome. CONCLUSION: This case report highlights the diverse neoplastic potential of nevus sebaceus and demonstrates the capacity of this hamartoma to develop aggressive tumors, such as sebaceous carcinoma. Prophylactic excision or at least close clinical surveillance for sudden development of new growths is warranted in all cases of nevus sebaceus.

Mohs micrographic surgery for angiolymphoid hyperplasia with eosinophilia.

BACKGROUND: Angiolymphoid hyperplasia with eosinophilia is a benign vascular proliferation that typically presents on the head and neck. Multiple treatment modalities have been proposed for angiolymphoid hyperplasia with eosinophilia, each with limited success or undesirable side effects. At this time, standard surgical excision is considered the treatment of choice but carries recurrence rates of 33% to 50%. OBJECTIVE: The objective was to present a case of angiolymphoid hyperplasia with eosinophilia successfully extirpated using Mohs micrographic surgery. METHODS: A 52-year-old woman presented with an ill-defined solitary plaque of angiolymphoid hyperplasia with eosinophilia within her left conchal bowl that enlarged despite conservative therapy with intralesional and topical corticosteroids. Mohs micrographic surgery using the fresh tissue technique and standard hematoxylin and eosin staining was performed. The characteristic histologic features of angiolymphoid hyperplasia with eosinophilia were readily identifiable on frozen sections and complete extirpation required two stages of micrographically controlled resection. RESULTS: Resection of angiolymphoid hyperplasia with eosinophilia of the conchal bowl with complete resolution of symptoms and no evidence of clinical recurrence 8 months after surgery. CONCLUSION: Given the high recurrence rates reported for standard excision, Mohs micrographic surgery with complete margin examination should be considered as a treatment option for angiolymphoid hyperplasia with eosinophilia, particularly for lesions with ill-defined margins or in locations where tissue sparing is desirable.

Cystic basal cell carcinoma or hidrocytoma? The use of an excisional biopsy in a histopathologically challenging case.

Basal cell carcinoma (BCC) has many histologic variants. These variants may show differentiation toward benign and malignant tumors of cutaneous appendages, especially hair follicles. Herein we describe a lesion that was clinically thought to be a BCC, but a superficial biopsy showed histologic features suggestive of an apocrine hidrocystoma. Because of some cytologic atypia, complete excision was recommended. The excision specimen revealed a cystic BCC. The importance of examining the entire neoplasm before reaching a final pathologic diagnosis is emphasized.