Chronic recurrent annular neutrophilic dermatosisL98.2

Chronic recurrent anular neutrophilic dermatosis - CRAND - was first described by Christensen et al. in 1989. This disease is usually considered a subtype of Sweet syndrome. It remains to be seen to what extent this disease can be evaluated as an entity.

Chronic recurrent anular neutrophilic dermatosis, CRAND for short, is described as a rare (<20 published cases), benign, sometimes painful, neutrophilic dermatosis. CRAND has also been associated with IgG k-type monoclonal gammapathy, rheumatoid arthritis and sarcoidosis (Philibert F et al. 2020).

This disease affects women after the age of 40.

CRAND is characterized by chronic, recurrent, also painful, localized, plaque-shaped, inflammatory skin lesions. The clearly infiltrated PLaque may be characterized by fine, even coarse lamellar scaling. The lesions develop within four weeks and grow centrifugally. Recurrent occurrence (within months to 1-5 years) is apparently characteristic. Recurrent lesions are morphologically identical to primary lesions and tend to be isotopic (recurrence in locoo).

The histological features are similar to those of Sweet's syndrome and are characterized by dermal oedema and dense neutrophil infiltrates in the upper and middle corium. No leukocytoclasia (Croci-Torti A et al. 2017).

1. A 41-year-old woman had been observing annular lesions on her forearms and décolleté for about 1 year. Examination revealed a 5 cm annular lesion on the right forearm and four similar neighboring lesions. The condition resolved spontaneously after 4 weeks. Three months of treatment with hydroxychloroquine 400 mg per day proved to be ineffective, as the disease recurred under this therapy. However, after two months of treatment with colchicine at a daily dose of 1 mg, the rash did not recur over an observation period of 10 years.
2. A 38-year-old woman was consulted because of recurrent ring-shaped erythema confined to the legs. Examination revealed a red, plaque-like lesion on the right leg with central scaling. The lesion had been present for three weeks. In both cases, histologic examination was reminiscent of Sweet's syndrome, but no inflammatory or neutrophilic syndrome or underlying systemic disease was detected.
3. A 50-year-old woman presented with ring-shaped plaques on the forearms that had been recurring for two years. Dermatologic examination revealed erythematous-edematous, painful and infiltrated plaques with centrifugal extension and fine central scales on the forearms. The patient had no fever. The rest of the physical examination was unremarkable. The blood tests were within the normal range. The skin biopsy confirmed the diagnosis of "Sweet syndrome" and showed edema in the superficial dermis and a dense inflammatory infiltration of neutrophils without accompanying vasculitis. The patient was treated with high-dose topical corticosteroids, which led to progressive regression of the lesions within a week. During the eight-year follow-up period, the patient experienced a recurrence of the same skin lesions on the forearms every year, which improved with the same topical treatment.

1. Christensen OB et al. (1989) Chronic recurrent annular neutrophilic dermatosis. An entity? Acta Derm Venereol 69:415-418.
2. Croci-Torti A et al. (2017) Dermatose neutrophilique annulaire récurrente chronique [Chronic recurrent annular neutrophilic dermatosis]. Ann Dermatol Venereol 144:362-367.
3. Foster AM et al. (2014) IL-36 promotes myeloid cell infiltration, activation, and inflammatory activity in skin. J Immunol 192:6053-6061.
4. Ma HL et al. (2010) Tumor necrosis factor alpha blockade exacerbates murine psoriasis-like disease by enhancing Th17 function and decreasing expansion of Treg cells. Arthritis Rheum 62:430-440.
5. Manaa L et al. (2022) Chronic recurrent annular neutrophilic dermatosis: A rare entity. Clin Case Rep 10:e05771.
6. Philibert F et al. (2020) Chronic recurrent annular neutrophilic dermatosis revealing sarcoidosis. JAAD Case Rep 6:285-288.
7. Salih Alj M et al. (2022) Associated Pyoderma Gangrenosum, Erythema Elevatum Diutinum, and Chronic Recurrent Annular Dermatosis: The Neutrophilic Disease Spectrum. Cureus 14:e21005.