Non‐infectious granulomatous dermatoses

Abstract

Summary Granulomatous dermatoses comprise a wide range of etiologically and clinically distinct skin diseases that share a common histology characterized by the accumulation of histiocytes include macrophages. While the pathogenesis of these disorders is not fully understood, the underlying mechanism is thought to involve a reaction pattern caused by an immunogenic stimulus. Antigen‐presenting cells and the effect of various cytokines play a key role. Our understanding of granulomatous reaction patterns has been advanced by insights drawn from observations of such reactions in patients on immunomodulatory therapy and in individuals with genetic immunodeficiency. Traditionally, a distinction is made between infectious and non‐infectious granulomatous dermatoses. The present CME article addresses granulomatous skin diseases for which there is no evidence of a causative infectious agent. Common representatives include granuloma annulare, necrobiosis lipoidica and cutaneous sarcoidosis. Granulomatous dermatoses may be part of the clinical spectrum of various systemic disorders or may be associated therewith. Some neoplastic disorders may mimic granulomatous dermatoses histologically. Given the pathogenetic diversity involved, the clinical presentation, too, is quite varied. Overall, however, each disorder is characterized by typical clinical features. The diagnosis always requires thorough clinicopathologic correlation. Treatment is preferably based on the underlying pathogenesis and frequently involves anti‐inflammatory agents. In most cases, however, there is insufficient study data. The dermal nature of these disorders frequently poses a therapeutic challenge, especially with respect to topical treatment.

A large congenital cutaneous tumor

“…The clinical and dermoscopic appearance of giant congenital JXG is distinctive, but the differential diagnosis may include vascular tumors (hemangioma or tufted angioma), malignant tumors (congenital dermatofibrosarcoma protuberans or rhabdomyosarcoma), and other conditions like granulomatous diseases (which also show histiocytes), solitary mastocytoma or juvenile nodular fasciitis [8][9][10][11].…”

Immune-Mediated Dermatoses in Patients with Haematological Malignancies: A Comprehensive Review

“…Granulomatous dermatoses comprise a wide range of clinically and aetiologically distinct disorders characterised by a granulomatous dermal inflammatory infiltrate predominantly composed of histiocytes and macrophages [221,222]. Albeit rare, associations with HMs have been found for various granulomatous dermatoses, including granuloma annulare, cutaneous sarcoidosis, interstitial granulomatosis and palisaded neutrophilic granulomatous dermatitis.…”

Ein großer kongenitaler kutaner Tumor

“…Das klinische und dermoskopische Erscheinungsbild des angeborenen Riesen-JXG ist charakteristisch, jedoch kann die Differenzialdiagnose vaskuläre Tumoren (kavernöse und kapilläre Hämangiome) und maligne Tumoren (angeborenes Dermatofibrosarcoma protuberans oder Rhabdomyosarkom) umfassen. Auch andere Krankheitsbilder wie granulomatöse Erkrankungen (die auch Histiozyten aufweisen), solitäre Mastozytome oder juvenile noduläre Fasziitiden sind möglich [8][9][10][11]. Zusammenfassend lässt sich sagen, dass es sich beim kongenitalen Riesen-JXG um einen seltenen gutartigen Tumor handelt, der bei der Differenzialdiagnose rötlich-gelber Läsionen, die von Geburt an vorhanden sind, in Betracht gezogen werden sollte.…”