Annular elastolytic giant cell granuloma associated with autoimmune hepatitis: Response to ciclosporin

A 47 year old Caucasian female with a background of type 2 diabetes mellitus, hypothyroidism, and autoimmune hepatitis presented with a painful, pruritic, papular eruption in a photosensitive distribution across the upper chest, neck, face, dorsal hands and forearms. On examination, lesions coalesced into annular plaques each with an active, raised margin and an atrophic, yellow centre. Histopathology demonstrated an absence of mucin and elastophagocytosis with giant cells engulfing dermal elastin fibres. These histopathological features favoured a diagnosis of annular elastolytic giant cell granuloma (AEGCG). The patient was managed ciclosporin monotherapy 125 mg twice daily (3 mg/kg/day). At 8 week review, there was a marked improvement in the physical appearance of the dermatosis as well as diminishing of symptoms such as itch and cutaneous pain. AEGCG is a rare inflammatory dermatosis typically affecting sun-exposed sites. It has been proposed that AEGCG is triggered by a solar induced elastolysis however other theories suggest it is a primary granulomatous disorder and not a photodermatosis. AEGCG appears to be aligned to an autoimmune diathesis, indicated by its frequent association with autoimmune conditions such as Hashimoto's thyroiditis, vitiligo, giant cell arteritis and, as in our patient, auto-immune hepatitis. Diabetes mellitus occurring concurrently with AEGCG has also been observed, again like our patient. Histopathological features which distinguish AEGCG from granuloma annulare include absent mucin, absent necrobiosis, giant cells with more nuclei, non-palisading granulomata and marked loss of elastic tissue. AEGCG is often unresponsive to standard therapies. The literature indicates varying responses to photo-protection, topical/systemic/intralesional corticosteroids, and oral medications such as methotrexate, hydroxychloroquine, and dapsone. Few case reports have also documented improvement with ciclosporin. In aggressive forms of AEGCG, as in our patient, treatment with ciclosporin may be an effective intervention and should be initiated early in the disease.