Suzanne M. Connolly

AAD/ACMS/ASDSA/ASMS 2012 appropriate use criteria for Mohs micrographic surgery: A report of the American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery

The appropriate use criteria process synthesizes evidence-based medicine, clinical practice experience, and expert judgment. The American Academy of Dermatology in collaboration with the American College of Mohs Surgery, the American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery has developed appropriate use criteria for 270 scenarios for which Mohs micrographic surgery (MMS) is frequently considered based on tumor and patient characteristics. This document reflects the rating of appropriateness of MMS for each of these clinical scenarios by a ratings panel in a process based on the appropriateness method developed by the RAND Corp (Santa Monica, CA)/University of California-Los Angeles (RAND/UCLA). At the conclusion of the rating process, consensus was reached for all 270 (100%) scenarios by the Ratings Panel, with 200 (74.07%) deemed as appropriate, 24 (8.89%) as uncertain, and 46 (17.04%) as inappropriate. For the 69 basal cell carcinoma scenarios, 53 were deemed appropriate, 6 uncertain, and 10 inappropriate. For the 143 squamous cell carcinoma scenarios, 102 were deemed appropriate, 7 uncertain, and 34 inappropriate. For the 12 lentigo maligna and melanoma in situ scenarios, 10 were deemed appropriate, 2 uncertain, and 0 inappropriate. For the 46 rare cutaneous malignancies scenarios, 35 were deemed appropriate, 9 uncertain, and 2 inappropriate. These appropriate use criteria have the potential to impact health care delivery, reimbursement policy, and physician decision making on patient selection for MMS, and aim to optimize the use of MMS for scenarios in which the expected clinical benefit is anticipated to be the greatest. In addition, recognition of those scenarios rated as uncertain facilitates an understanding of areas that would benefit from further research. Each clinical scenario identified in this document is crafted for the average patient and not the exception. Thus, the ultimate decision regarding the appropriateness of MMS should be determined by the expertise and clinical experience of the physician.

Linear IgA dermatosis: Association with malignancy

We report the association of linear IgA dermatosis with hematologic malignancy (chronic lymphatic leukemia and plasmacytoma) in two patients. Ten documented cases of linear IgA dermatosis and internal malignancy have been reported in the literature. The possible association of malignancy and linear IgA dermatosis is discussed.

Prurigo Nodularis: A Reappraisal of the Clinical and Histologic Features

Study of 14 patients with prurigo nodularis revealed that clinically the lesions were multiple, verrucous, and discrete and were set in normal‐appearing skin. Microscopically, the lesions showed a vascular acanthomatous mass. The dermal nerves showed a tendency toward hyperplasia in parallel with the epidermal and vascular changes, but no evidence of neuroma formation was seen. Electron microscopic examination showed disorganization of the normal pattern of the myelin sheaths and axons of the dermal papillary nerves. The presence of epidermal mast cells was noted in the biopsy specimens of prurigo nodules from eight of the 14 patients.

The vascular calcification-cutaneous necrosis syndrome

BACKGROUND Although medial calcification of larger elastic arteries in chronic kidney failure and with advancing age is relatively common, calcification of the cutaneous vascular system is rare. OBJECTIVE Our purpose was to describe three patients with the vascular calcification-cutaneous necrosis syndrome and review the cause, clinical and pathologic features, and treatment of this syndrome. METHODS We describe three patients with ischemic necrotic ulcers and underlying cutaneous vascular calcification. The clinical setting was abnormal calcium metabolism from either chronic kidney failure or excessive vitamin D intake. RESULTS The clinical findings in all patients consisted of multiple tender livedoid nodules and ulcerative plaques on the thighs and legs, which developed in the setting of abnormal calcium metabolism from either chronic kidney failure or excessive vitamin D intake. Histologic study demonstrated vascular calcification. Although this syndrome usually has a chronic course with significant morbidity and mortality, subtotal parathyroidectomy followed by kidney transplantation resulted in complete resolution in one of our patients. CONCLUSION The clinical and histopathologic findings in the vascular calcification-cutaneous necrosis syndrome are unique. The pathogenesis is likely multifactorial. Treatment for the skin lesions is largely supportive.

AAD/ACMS/ASDSA/ASMS 2012 Appropriate Use Criteria for Mohs Micrographic Surgery: A Report of the American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery

&NA; The appropriate use criteria process synthesizes evidence‐based medicine, clinical practice experience, and expert judgment. The American Academy of Dermatology in collaboration with the American College of Mohs Surgery, the American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery has developed appropriate use criteria for 270 scenarios for which Mohs micrographic surgery (MMS) is frequently considered based on tumor and patient characteristics. This document reflects the rating of appropriateness of MMS for each of these clinical scenarios by a ratings panel in a process based on the appropriateness method developed by the RAND Corp (Santa Monica, CA)/University of California–Los Angeles (RAND/UCLA). At the conclusion of the rating process, consensus was reached for all 270 (100%) scenarios by the Ratings Panel, with 200 (74.07%) deemed as appropriate, 24 (8.89%) as uncertain, and 46 (17.04%) as inappropriate. For the 69 basal cell carcinoma scenarios, 53 were deemed appropriate, 6 uncertain, and 10 inappropriate. For the 143 squamous cell carcinoma scenarios, 102 were deemed appropriate, 7 uncertain, and 34 inappropriate. For the 12 lentigo maligna and melanoma in situ scenarios, 10 were deemed appropriate, 2 uncertain, and 0 inappropriate. For the 46 rare cutaneous malignancies scenarios, 35 were deemed appropriate, 9 uncertain, and 2 inappropriate. These appropriate use criteria have the potential to impact health care delivery, reimbursement policy, and physician decision making on patient selection for MMS, and aim to optimize the use of MMS for scenarios in which the expected clinical benefit is anticipated to be the greatest. In addition, recognition of those scenarios rated as uncertain facilitates an understanding of areas that would benefit from further research. Each clinical scenario identified in this document is crafted for the average patient and not the exception. Thus, the ultimate decision regarding the appropriateness of MMS should be determined by the expertise and clinical experience of the physician.

Pseudosclerodermatous Panniculitis After Irradiation: An Unusual Complication of Megavoltage Treatment of Breast Carcinoma

An unusual edematous and indurated erythema developed in four patients with breast carcinoma 1 to 6 months after conservative surgical treatment and irradiation. The radiation therapy consisted of megavoltage x-ray photon with or without either electron beam or iridium-192 interstitial boost. Several tissue biopsy specimens revealed pronounced lymphocytic dermal and fat inflammation in conjunction with focal areas of plasma cells. The connective tissue bundles were enlarged and hyalinized. Macrophages and isolated giant cells were noted in the dermis. One biopsy specimen showed elastic tissue in giant cell cytoplasm. No mucin, fibrin, formation of cysts, or calcification was present. Lipophages and hyaline connective tissue replaced some fat lobules. The radiation-induced changes of dilated and hyalinized blood vessels, endothelial cell hyperplasia, fibrosis associated with involution of epidermal appendages, and fibroblasts were present. This combination of radiation-related and inflammatory pathologic changes is unusual and emphasizes the remarkable qualities of this rare reaction. The clinical differential diagnoses of recurrent carcinoma, cellulitis, and connective tissue disease can be excluded by reviewing the pathologic characteristics.

An association between C1 esterase inhibitor deficiency and lupus erythematosus: Report of two cases and review of the literature

Two patients who had lupus erythematosus and C1 esterase inhibitor deficiency are described. The data on eleven previously reported cases are reviewed and summarized with those from our own cases. Early complement component depletion secondary to C1 esterase inhibitor deficiency may predispose to the development of lupuslike syndromes by impairing the ability of the organism to handle foreign antigen. Further study of this unique group of patients may provide insight into the pathogenesis of immune complex disease.

Clinicopathologic spectrum of specific cutaneous lesions of disseminated coccidioidomycosis

BACKGROUND Disseminated coccidioidomycosis merits greater attention because the number of persons living and traveling in endemic areas is increasing. OBJECTIVE Our purpose was to study the clinical and histopathologic findings in patients with specific cutaneous disseminated coccidioidomycosis. METHODS In six patients with specific skin lesions of disseminated coccidioidomycosis, the diagnosis was confirmed by identification of the organism in tissue or by positive results of tissue culturing. RESULTS Clinical lesions included solitary granulomatous plaques in two patients and multiple papular, nodular, or pustular lesions in four patients, two of whom also had subcutaneous abscesses. Identifying organisms directly in tissue was possible in only 8 of 17 biopsy specimens and in five of six patients. The histopathologic features showed various degrees of three primary patterns: (1) abscess formation with necrosis, (2) epithelial hyperplasia and granuloma formation with microabscesses, and (3) vascular and perivascular proliferative and inflammatory cell reactions at times suggesting vasculitis. Tissue eosinophilia, present in all patients, was striking in two (eosinophilic abscess formation) and notable in another (vascular inflammation with eosinophilia). CONCLUSION Cutaneous manifestations of disseminated coccidioidomycosis may be more common and varied than usually recognized.

Bronopol allergic contact dermatitis

Bronopol (2‐bromo‐2‐nitropropane‐1, 3‐diol) is an antimicrobial widely used as a preservative, primarily in cosmetic formulations. Analysis of patch tests performed on our patients revealed an incidence of 12.5% relevant positive results to 0.5% and/or 0.25% bronopol. This result reflects a history of prolonged use of bronopol‐containing lubricants in our referral population of patients with different types of severe, extensive dermatitis. Contact sensitization to bronopol in this population is probably facilitated by abnormal cutaneous barrier function. Our findings emphasize the need for further clinical study of the potential for bronopol to produce contact sensitivity, and suggest caution with regard to its use in patients with dermatitis.

Histopathologic Features of the l-Tryptophan-Related Eosinophilia-Myalgia (Fasciitis) Syndrome

Study of 18 biopsy specimens in 11 patients with L-tryptophan-related eosinophiliamyalgia (fasciitis) syndrome showed hyaline sclerodermoid changes. Dermal scleroderma was found in eight of nine punch biopsy specimens and eight of nine excisional biopsy specimens. Fascial scleroderma was found in eight excisional biopsy specimens. One specimen obtained by excision had no fascia present. Eleven biopsy specimens showed edema of the dermis, and 13 showed dilated lymphatic structures; thus, the clinical picture of edematous sclerosis was confirmed. Mucinous fasciitis was present in five excisional biopsy specimens, in conjunction with a large number of macrophages in four. Dermal mucinosis was present in 11 biopsy specimens. Lymphocytic and macrophage inflammation was minimal in 14 biopsy specimens and pronounced in only 4. Plasma cells were present in eight cases. Eosinophils were present in substantial numbers in three biopsy specimens and only occasionally in four. Eosinophilic spongiosis was observed in one patient. Lymphocytic inflammation was noted around a single muscle spindle and around large nerve trunks in three patients. No relationship was established between these pathologic features and the duration or dose of tryptophan, prednisone treatment, or duration of symptoms. Pathologic features of the L-tryptophan syndrome consist of hyaline sclerodermoid collagen in the dermis, the septa, and the fascia. Edema, focal mucinosis, and macrophage inflammation may be features that identify this event.