Wong-type dermatomyositis: a mimic of many dermatoses.

Wong-type dermatomyositis (DM) is a rare variant characterized by keratotic follicular papules that may mimic pityriasis rubra pilaris. Histopathologic examination shows follicular and non-follicular epidermal invaginations filled with keratin. The diagnosis is often delayed. Twenty-four cases of Wong-type DM have been reported thus far in the literature. Herein, we report the clinical and histopathologic findings of three additional cases in order to raise awareness of the disorder.

Cutaneous metastasis as the presenting sign of papillary thyroid carcinoma.

Cutaneous metastasis of visceral tumors accounts for 2% of skin tumors. We report the case of a 71-year-old male with a smoking history who presented to dermatology department with a violaceous nodule of the right sideburn skin. The lesion was interpreted as an adenocarcinoma that was completely excised and was suspicious for a metastasis. There was a recommendation for additional work-up. At a different institution, a positron emission tomography scan showed a left hilar mass and uptake in the right thyroid. He was then referred to our hospital for tissue diagnosis. Mediastinoscopy with biopsy of the left hilar mass showed metastatic follicular thyroid carcinoma. Subsequently, a thyroid fine needle aspirate showed suspicion for malignancy with similar morphology. Thyroidectomy and central neck dissection showed right thyroid papillary carcinoma extending to one margin and involving the lymph nodes. The left hilar metastasis mass resection showed similar lymph node findings. A re-review of the sideburn excision revealed similar histopathology to the thyroid and mediastinal resection. This case illustrates the opportunity of considering metastatic thyroid carcinoma to skin even in cases which lack the classic cytologic and architectural features of papillary thyroid carcinoma follicular variant.

A case of pemphigus herpetiformis occurring in a 9-year-old boy.

Pemphigus herpetiformis (PH) is a rare autoimmune vesiculobullous disease. It clinically mimics dermatitis herpetiformis and has immunofluorescent findings typical of pemphigus. PH occurs in adults more commonly than children and is often effectively treated with dapsone. We report a case of PH occurring in a 9-year-old boy that was refractory to dapsone and to various other steroid-sparing medications but resolved with methotrexate.

Hereditary mucoepithelial dysplasia: unique histopathological findings in skin lesions.

Hereditary mucoepithelial dysplasia (HMD) is a rare, autosomal dominantly inherited, multisystem disease thought to be caused by abnormalities in desmosomes and gap junctions. HMD affects the skin, mucosae, hair, eyes and lungs. Nearly all patients clinically display the triad of non-scarring alopecia, well-demarcated mucosal erythema and erythematous intertriginous plaques. Although histopathological findings of mucous membrane epithelium have been well characterized, only three reports have described histopathologic findings in the skin. We report a case with unique histopathological findings not previously reported in HMD.

Azathioprine-induced EBV-positive mucocutaneous ulcer.

Epstein-Barr virus (EBV)-positive mucocutaneous ulcer was recently described as a clinicopathologic entity occurring secondary to iatrogenic or age-related immune suppression. The histopathology of EBV-positive mucocutaneous ulcer reveals a polymorphous infiltrate including atypical large B-cells and Reed-Sternberg-like cells which are CD20-positive, CD30-positive and EBV-positive. The disorder follows an indolent and self-limited course. We report a case of EBV-positive mucocutaneous ulcer secondary to prolonged use of azathioprine for the treatment of pemphigoid and highlight the need for recognition of this disorder by dermatopathologists and dermatologists.

Annular lipoatrophy of the ankles: case report and review of the literature.

Annular lipoatrophy of the ankles also termed "annular atrophic connective tissue panniculitis of the ankles,""annular atrophy of the ankles," and "atrophic annular panniculitis of the ankles," is an inflammatory panniculitis that results in lipoatrophy with a predilection for the ankles. The disorder is seen predominantly in female children and demonstrates a mixed lobular panniculitis. It is likely a disorder of autoimmune etiology, supported by the findings of concurrent autoimmune disorders in several patients. We report a case of a 6-year-old female with annular lipoatrophy of the ankles associated with Hashimoto thyroiditis and review the epidemiology and proposed pathogenesis of annular lipoatrophy of the ankles.

Acquired idiopathic generalized anhidrosis: case report.

We report a case of acquired idiopathic generalized anhidrosis (AIGA) in a 56-year-old white woman. Acquired idiopathic generalized anhidrosis is an exceedingly rare group of heterogeneous disorders that has been almost exclusively reported in young Japanese males. Our case is unique in that AlGA may be underrecognized in this patient population.

Amelanotic lentigo maligna: a report of three cases and review of the literature.

BACKGROUND: Amelanotic lentigo maligna is not clinically suspected and is often mistaken for a basal cell carcinoma, squamous cell carcinoma, or dermatitis. OBJECTIVE: Our objective was to review previously reported cases of amelanotic lentigo maligna and compare them with our 3 cases. METHODS: The clinical presentation and histologic findings of 3 new cases are described and compared with those in the literature. RESULTS: The index of suspicion for amelanotic lentigo maligna is extremely low. No reported cases have been diagnosed clinically. None of our 3 cases was suspected. LIMITATIONS: Only three cases were reviewed. CONCLUSION: A high degree of clinical and histologic suspicion is required to make the diagnosis of this clinically nondescript neoplasm.

The psychiatric profile of patients with psychogenic excoriation.

BACKGROUND: Psychogenic excoriation describes patients who excoriate without apparent primary skin abnormality. OBJECTIVE: This study compares the psychiatric profile of patients with psychogenic excoriation with a control group. METHODS: Fifty consecutive patients with psychogenic excoriation were matched with 50 control patients and given a questionnaire related to psychiatric history. RESULTS: Study and control groups differed significantly in relation to psychiatric comorbidity, use of psychotropic drugs, history of depression, and history of bipolar disorder. Psychogenic excoriation was not associated with personal history of other psychiatric disorders or family history of psychiatric comorbidity, except alcoholism. LIMITATIONS: Nonpsychiatrists may have made the psychiatric diagnoses. CONCLUSION: Psychogenic excoriation is significantly associated with psychiatric comorbidity, especially depression and bipolar disorder. Awareness by the patient of a link between the psychiatric disorder and the cutaneous condition may help patients in dealing with the distress caused by the unknown nature of the excoriating behavior.

Macular lymphocytic arteritis: a unique benign cutaneous arteritis, mediated by lymphocytes and appearing as macules.

BACKGROUND: Macular arteritis (MA) is a term coined for a cutaneous vasculitis that manifests as multiple macules that favor the lower extremities and that reveal lymphocytic inflammation of an artery in the deep dermis and subcutaneous fat. METHODS: We describe the demographic, clinical, histologic, and laboratory findings in three new cases and review seven previously reported cases of MA, and discuss the nosology of MA. RESULTS: MA affects predominantly females (70% or 7/10) and individuals of African-American race (50%). Eight patients had hyperpigmented patches, one had hypopigmented macules, and one had erythematous macules. The lower extremities were involved in 100% of cases, followed by the upper extremities (44%). Light microscopic changes are those of a medium-vessel lymphocytic arteritis. Investigations for possible causes are non-revealing. CONCLUSION: We conclude that MA is a primary idiopathic cutaneous lymphocytic arteritis that is limited to the skin.

Macular arteritis mimicking pigmented purpuric dermatosis in a 6-year-old caucasian girl.

A 6-year-old Caucasian girl presented with a 4-month history of a mildly pruritic, brown and pink, focally petechial macules on the posterior aspect of her left leg, clinically consistent with a pigmented purpuric dermatosis. Biopsy, however, revealed lymphocytic inflammation with occlusion of the lumen of a small artery at the dermal-subcutaneous junction, characteristic of macular arteritis. This is a recently described entity that is not known to be associated with systemic disease, and remains stable over years without treatment. So far, it has not been reported in a Caucasian patient. We review the clinical and histologic features, as well as the laboratory evaluation of this case, and the previously reported cases.

Skin manifestations of inflammatory bowel disease.

Ulcerative colitis (UC) is an inflammatory disorder of the colon that is associated with several extraintestinal manifestations in multiple organs. Several mucous membrane and skin disorders occur in patients with UC. These disorders are not unique to UC and often occur secondary to other causes or in the absence of an apparent cause. One or more such disorders may occur together in association with UC. Mucous membrane and skin disorders may antedate, occur with, or postdate the onset of UC. The dermatologist plays an important role in suspecting the diagnosis of UC that presents with associated mucous membrane or skin disorders. This review covers the clinical presentation, differential diagnosis, workup, and management of selected mucocutaneous manifestations in UC.