The utility and challenges of histopathologic evaluation in the diagnosis of nonmalignant skin ulcers.

Histopathologic evaluation of cutaneous ulcers is indicated when the clinical diagnosis is unclear or when ulcers have not responded to standard of care. Many nonmalignant skin ulcers lack specific histologic findings on biopsy and pose a diagnostic challenge. While the usefulness of skin biopsies to diagnose underlying malignancy in ulcerated lesions has been demonstrated in previous studies, their utility in the diagnosis of ulcers of other etiologies has not been reported. We conducted a retrospective study of 45 nonmalignant ulcer biopsies in a 3-year period to compare the histologic diagnosis with the final diagnosis. Additionally, we assessed the diagnostic concordance among three blinded dermatopathologists when reviewing these cases. The leading histologic diagnosis from each of the three observers agreed with the final clinical diagnosis, on average, for 29.6% of the cases (average pairwise kappa = 0.15). Inflammatory ulcers had the lowest concordance between the observers and final diagnosis with an average of 26.0% of cases (average pairwise kappa = 0.06). The observers agreed with each other for 35.6% of the cases (Fleiss' kappa = 0.32). The highest agreement among observers was in the vascular/vasculopathic category (50%, Fleiss' kappa = 0.44). Our results indicate that skin biopsies alone are useful in the evaluation of nonmalignant ulcers to rule out other conditions (e.g. neoplasm) but frequently not sufficient to establish a definitive diagnosis. Additional clinicopathologic correlation is necessary in the final assessment of nonmalignant ulcers to determine the diagnosis. Future research endeavors should explore alternative approaches to more efficiently diagnose nonmalignant skin ulcers.

Carcinoma Erysipeloides From Metastatic Cutaneous Squamous Cell Carcinoma Initially Mistaken for Intralymphatic Histiocytosis.

Intralymphatic histiocytosis is a rare dermatologic disorder, commonly associated with inflammatory disorders and rarely malignancy. Carcinoma erysipeloides (CE) is a rare pseudoinflammatory cutaneous eruption that resembles soft -tissue infections as result of intralymphatic metastasis and subsequent lymphatic obstruction. Breast carcinoma represents most of the CE cases, but rarely other malignancies are involved. This report discusses a patient with a history of cutaneous squamous cell carcinoma (SCC) of the temple, who was initially diagnosed with intralymphatic histiocytosis located on his upper extremity, resistant to treatment. Further dermatologic and pathologic review later revealed metastatic SCC restricted to the dermal lymphatics, creating a CE reaction, initially obscured by intralymphatic histiocytes. This case highlights the difficulty in diagnosing metastatic carcinoma when the malignant cells are accompanied by a dense histiocytic infiltrate. The case demonstrates a rare presentation of CE due to metastatic cutaneous SCC and highlights the need for persistent investigation when confronted with nonconforming pathology.

Nipple leiomyoma: A rare neoplasm with a broad spectrum of histologic appearances.

Cutaneous leiomyomas are rare benign smooth-muscle tumors. These lesions are distinguished based on their cell of origin and are subclassified as pilar leiomyoma, angioleiomyoma, and genital-type leiomyoma. Nipple leiomyoma is the least common genital-type leiomyoma, arising from the dartoic muscle cell of the nipple. Histologic examination of the lesion is necessary for definitive diagnosis, and these uncommon tumors can pose a diagnostic challenge. We describe herein a series of six nipple leiomyomas with a spectrum of histologic appearances.

Generalized essential telangiectasia.

A 56-year-old woman presented with multiple, erythematous cutaneous vessels that blanched with diascopy and were symmetrically distributed over both lower and upper extremities. The lesions had been present for approximately 6 years and were asymptomatic. Generalized essential telangiectasia (GET) is an idiopathic syndrome of widespread, asymptomatic telangiectases of unknown cause. In our patient, an extensive laboratory evaluation showed low levels of circulating vitamin C as well as the presence of urinary matrix metalloproteinases. The relevance of these abnormal findings is unclear. No uniformly effective treatment exists for GET. Our patient was started on oral doxycycline and was referred for laser therapy.

Eruptive xanthomas.

A 26-year-old woman presented for evaluation of multiple, red-yellow papules that had been appearing over the course of 2 months on the extensor surfaces of the upper extremities, dorsal aspects of the hands, thighs, and buttocks. Serum lipid evaluation showed hypertriglyceridemia in a patient with a recent history of pancreatitis. A diagnosis of eruptive xanthomas was made based on clinical, laboratory, and histopathologic findings.

White sponge nevus.

A 33-year-old-man presented with a 13-year history of asymptomatic, white, folded, soft, poorly-demarcated, diffuse plaques bilaterally on his buccal mucosae and lateral surfaces of his tongue. There is no family history of similar lesions. The physical examination and histopathologic findings were consistent with a diagnosis of white sponge nevus. This rare disorder is typically inherited; however, as in this case, there have been a few other cases reported without a familial background.

Palisaded neutrophilic and granulomatous dermatosis.

A 22-year-old woman with mixed connective-tissue disease presented with a 5-month history of recurrent episodes of tender, erythematous papules, nodules, and edematous plaques on the upper extremities and thighs. Cutaneous lesions occurred in the setting of livedo reticularis. A biopsy specimen showed interstitial and perivascular inflammation with lymphocytes, macrophages, neutrophils, nuclear dust, collagen alteration, extravasated erythrocytes, and fibrin within small superficial blood vessels. These changes were consistent with a diagnosis of palisaded neutrophilic and granulomatous dermatosis, which is a rare entity that includes a combination of a neutrophilic infiltrate, abnormal or altered collagen, granuloma formation, and leukocytoclastic debris in the context of an immune-mediated collagen vascular or systemic disease. The underlying mechanism remains poorly understood. Treatment is limited, and resolution of lesions typically occurs within several months to years.

Interstitial granulomatous dermatitis.

A 59-year-old woman with arthritis presented to the Skin Institute of New York with a 2-month history of asymptomatic, small, skin-colored papules that erupted symmetrically on the chest, back, and proximal extremities. Histopathologic examination of a biopsy specimen showed findings of interstitial granulomatous dermatitis. Clinical correlation suggested a diagnosis of interstitial granulomatous dermatitis with arthritis. No change in the lesions resulted from application of clobetasol 0.05 percent ointment to the affected areas.

Granuloma annulare, patch type.

A 64-year-old man was referred to the Bellevue Hospital Center Dermatology Clinic for evaluation of an asymptomatic eruption on his left inner arm, which had been present for 4 months and was unresponsive to topical anti-fungal therapy. One month after the initial eruption, 2 similar, asymptomatic lesions appeared on the right inner arm. The lesions were slowly expanding. A biopsy specimen from the left medial arm was consistent with interstitial granuloma annulare. The patient's clinical presentation was consistent with patch-type granuloma annulare. He was treated with a mid-potency topical glucocorticoid twice daily for 4 weeks without benefit. Since the eruption was asymptomatic, treatment was discontinued.

Extragenital lichen sclerosus et atrophicus.

A 76-year-old woman with a history of eczematous dermatitis presented with a 9-month history of a pruritic, eczematous eruption of the trunk. This eruption responded to topical glucocorticoids but recurred on discontinuation of treatment on multiple occasions. A biopsy specimen showed extragenital lichen sclerosus et atrophicus. The diagnosis of extragenital lichen sclerosus is reviewed with a comparison to classic lichen sclerosus in terms of clinical features, histopathologic characteristics, and treatment approaches.

Chronic actinic dermatitis.

A 71-year-old man presented with a six-year history of a pruritic, erythematous, blistering eruption of the face, chest, and arms. Clinical findings, histopathologic features, and phototests were consistent with a diagnosis of chronic actinic dermatitis. The patient also had contact allergy and photocontact allergy to multiple allergens. A discussion of chronic actinic dermatitis is presented.

Striate palmoplantar keratoderma (Brunauer-Fohs-Siemens syndrome).

A 64-year-old man presented with focal hyperkeratotic plaques on the fingers, palms, and soles. Histopathologic and electron microscopic results were consistent with striate palmoplantar keratoderma. Treatment with topical keratolytics was unsuccessful. Striate palmoplantar keratoderma or Brunauer-Fohs-Siemens syndrome is an autosomal dominant condition that presents with linear hyperkeratosis on the palms and fingers and focal plaques on the plantar aspects of the feet. Histopathologic features include hyperkeratosis, hypergranulosis, and acanthosis with no epidermolysis. Electron microscopic examination shows diminished desmosomes, clumped keratin filaments, and enlarged keratohyalin granules. The syndrome has been linked to mutations in desmoglein 1, desmoplakin, and keratin 1. Treatment may include keratolytics, oral retinoids, and surgical debridement.

Lymphangioma circumscriptum.

A 54-year-old man presented for evaluation of multiple 1- to-4-mm, clear, pink, red, and black vesicles overlying hyperpigmented and erythematous indurated plaques on his right thigh and extending to his right inguinal fold. Clinical presentation and histopathologic features were consistent with lymphangioma circumscription. Lymphangioma circumscriptum is the most common cutaneous form of lymphangioma. Typical skin findings are multiple clear vesicles that may appear pink, red, or black. The first-line treatment option is surgical excision, although other modalities, such as laser therapy and sclerotherapy, have also been used successfully.

Sweet syndrome (acute febrile neutrophilic dermatosis) associated with pulmonary coccidioidomycosis.

BACKGROUND: Sweet syndrome (acute febrile neutrophilic dermatosis) may arise in association with a variety of underlying systemic diseases. Only 1 case of coccidioidomycosis-associated Sweet syndrome has previously been reported. OBSERVATIONS: We describe 2 patients who developed Sweet syndrome during the onset of acute pulmonary coccidioidomycosis. Systemic antifungal therapy was given in both cases. Respiratory symptoms and skin lesions resolved within 5 weeks. CONCLUSIONS: Sweet syndrome may be a presenting feature of coccidioidomycosis. Recognition of the underlying pulmonary infection is important so that inappropriate treatment with systemic corticosteroids can be avoided.