Faculty Bibliography

Vegetative pyoderma gangrenosum is a rare, superficial variant of pyoderma gangrenosum that is more commonly found on the trunk as single or multiple, non-painful lesions. There is typically no associated underlying systemic disease. Compared to classic pyoderma gangrenosum, vegetative lesions are more likely to heal without the use of systemic glucocorticoids, although up to 39% of patients required a short course of prednisone in a review of 46 cases. Treatments for vegetative pyoderma gangrenosum include topical and intralesional glucocorticoids, minocycline or doxycycline, dapsone, colchicine, and, rarely, alternative steroid-sparing immunosuppressants. We present a case of multiple vegetative pyoderma gangrenosum lesions arising in prior surgical sites in a patient found to have IgA monoclonal gammopathy and abnormal urinary protein electrophoresis.

A 55-year-old woman presented after a four-year history of impetiginized eczema that was distributed over her face, scalp, chest, and back and a recent diagnosis of breast cancer. A skin biopsy specimen for direct immunofluorescence demonstrated intercellular IgG4. Anti-desmoglein 1 and 3 antibodies were present. Indirect immunofluorescence testing was positive on monkey but not rat esophagus. These findings were consistent with a diagnosis of pemphigus foliaceus in association with a malignant condition. After many years of the use of topical glucocorticoids and oral antibiotics, the patient's disease was ultimately managed with methotrexate and a small dose of prednisone. Increasing data supports a role for a steroid-sparing effect of methotrexate in pemphigus vulgaris and likely pemphigus foliaceus.

Palmoplantar lichen planus (PPLP) is an uncommon variant of lichen planus that affects the palms and soles. Clinical findings are varied although they have been conceptualized into two large groupings, an erythematous scaly pattern and a hyperkeratotic pattern. Histopathologic features are those of classic LP. We present a case of PPLP that improved with methotrexate after failing treatment with acitretin.

Skin metastases from visceral malignancies have been well documented in the literature, and may be the presenting sign of an occult internal malignancy. Lung cancer in particular is a relatively common cause of skin metastases, which are considered a poor prognostic sign. Rarely, patients with lung cancer develop a second primary lung cancer that may require a novel chemotherapeutic regimen. The frequency of second primary malignancies presenting with metastatic skin lesions is not documented in the literature. We present a case of a 50-year-old man with a history of metastatic lung adenocarcinoma who was referred for evaluation of a nodule overlying his right mandible, which had been progressively enlarging for two weeks. Biopsy demonstrated metastatic squamous cell carcinoma. Subsequent CT-guided biopsy of a left retroperitoneal lymph node was conducted and notable for squamous cell carcinoma. Therefore, this patient's skin lesion was the presenting sign of a second primary visceral tumor, likely originating in the lung. We present this case to raise clinical awareness of the rare phenomenon that cutaneous metastasis may be the first sign of a visceral cancer, even in the setting of a previous distinct primary malignancy

J Drugs Dermatol 2014;13(10):1277-1279.

IMPORTANCE Medications as well as chemical and food exposures have been causally linked to the development of pigmented purpuric dermatosis (PPD). We describe herein what is to our knowledge the first reported case of isotretinoin-induced PPD. OBSERVATIONS A woman in her 30s presented with purpura annularis telangiectodes of Majocchi on the lower extremities 2 months after initiating isotretinoin for the treatment of refractory nodulocystic acne. CONCLUSIONS AND RELEVANCE We believe isotretinoin was the most likely causative agent in this case because the lesions began after initiation of isotretinoin treatment and resolved shortly after its termination, and the pathologic findings were consistent with other described cases of drug-induced PPD. The lesions have continued to fade, and no new lesions have developed in a 3-month follow-up period. Drug-induced PPD is distinct from idiopathic PPD, and it is important to consider isotretinoin as a potential inciting agent.

Morphea and lichen sclerosus et atrophicus (LSA) have similar clinical presentations. Reports of patients with overlapping clinical and histopathologic features of both conditions have led some to speculate that they may represent different presentations along the same disease spectrum. It has been postulated that there is a common etiologic agent, which may involve autoimmunity, response to trauma, or infection. The link between Borrelia infection and both morphea and LSA has been widely studied but remains controversial. We present a case of a patient with lesions characterized by overlapping features of morphea and LSA with rapid decrease in joint mobility.

We present a 21-year-old primigravida woman with a several-week history of pruritic, edematous, targetoid plaques that appeared initially on the abdomen, flanks, and legs and that progressed to involve the inner aspects of the upper arms and lateral aspects of the chest. The histopathologic findings showed perivascular and interstitial dermatitis with eosinophils and vacuolar changes with linear C3 deposition at the basement-membrane zone on direct immunofluorescence study. A diagnosis of pemphigoid gestationis was made. Pemphigoid gestationis is a rare, bullous dermatosis of pregnancy that may be associated with prematurity and small-for-gestational age birth weights. The diagnosis is often made with direct immunofluorescence studies of perilesional skin. Oral glucocorticoids remain the gold standard of therapy in moderate-to-severe cases. The edematous papules and plaques of pemphigoid gestationis may be particularly difficult to distinguish from polymorphic eruption of pregnancy; therefore, immunofluorescence studies are prudent. Prompt recognition and appropriate management may reduce morbidity of this disease, which often recurs with subsequent pregnancies.

A 35-year-old man initially was referred for management of recalcitrant urticaria. Owing to his long history of arthritis and sensorineural hearing loss, genetic testing was performed. The test showed a D305N heterozygous mutation in the NLRP3 gene, which is consistent with the diagnosis of Muckle-Wells syndrome. We discussed the rationales behind the use of the interleukin-1 antagonist anakinra in this autoinflammatory disorder.

A 57-year-old woman with systemic lupus erythematosus and Sjogren syndrome presented with blue-grey hyperpigmentation of the face, upper back, and dorsal aspects of the feet after seven years of therapy with hydroxychloroquine. We present an unusual case of drug-induced hyperpigmentation.