Hand-Schuller-Christian disease with secondary cutaneous involvement

Abstract

Summary A middle‐aged Arab lady presented with typical features of Hand‐Schuller‐Christian disease. In addition to diabetes insipidus and pulmonary fibrosis there was widespread bony involvement. Direct extension had occurred from the temporoparietal bone to the overlying skin with subsequent necrosis and ulceration. Such secondary cutaneous invasion has not been described previously.

Histiocytosis X in an adult mimicking pyoderma gangrenosum*

“…Frank ulcers in non-flexural areas are rare and occur late in the course of the disease. Goodfellow, Cream & Seed (1982) have reported a 31-year-old man with facial ulcers whilst Zachary & Macdonald (1983) described a middle-aged woman with temporoparietal ulceration. Both patients had chronic progressive histiocytosis X with extensive systemic involvement including pulmonary fibrosis and diabetes insipidus.…”

Aggressive histiocytic disorders that can involve the skin

“…13 Cutaneous lesions, which are present in approximately one third of patients, can be papulonodular, granulomatous, or seborrheic dermatitise like. 15 Eosinophilic granuloma is the localized, benign form of LCD and rarely produces cutaneous lesions. Eosinophilic granuloma presents in older children and adults, and the granulomatous lesions most often affect bone.…”

Cutaneous Hematopathology

“…Patients with Letterer-Siwe disease have extensive cutaneous lesions and classically resemble seborrheic dermatitis with involvement of the scalp, face, trunk, and perineum (Zachary and MacDonald 1983 ). Hand-Schuller-Christian disease has papulonodular, granulomatous, or seborrheic dermatitis-like lesions (Zachary and MacDonald 1983 ).…”

“…Hand-Schuller-Christian disease has papulonodular, granulomatous, or seborrheic dermatitis-like lesions (Zachary and MacDonald 1983 ). In Hashimoto-Pritzker disease, lesions are seen at birth and are widespread reddish-brown nodules that regress (Divaris et al 1991 ).…”

“…In Hashimoto-Pritzker disease, lesions are seen at birth and are widespread reddish-brown nodules that regress (Divaris et al 1991 ). LCH can also present with papules, pustules, vesicles, petechiae, or purpura (Zachary and MacDonald 1983 ;Stein et al 2001 ;Park et al 2012 ).…”