Eosinophilic and Neutrophilic Dermatoses

Eosinophilic pustular folliculitis
(Ofuji’s disease)
(Figure 3.44C)

Erythematous follicular papules, pustules, and plaques with annular or serpiginous pattern, occurring in recurrent crops; typically on face in men, associated pruritus

Histology: ↑↑ eosinophils around follicles, exocytosis of eosinophils and lymphocytes into follicular epithelium

Treatment: indomethacin (first line), dapsone, phototherapy, oral corticosteroid or minocycline (topical steroid if mild)

Eosinophilic folliculitis
(AIDS-associated)
(Figure 3.44B)

Follicular papules erupting over face, ± scalp and upper trunk; associated with significant pruritus and ↓↓ CD4 count

Histology: similar to Ofuji’s disease

Treatment: antiretroviral therapy to ↑ CD4 count, phototherapy, topical corticosteroid with oral antihistamine

Angiolymphoid hyperplasia with eosinophilia
(Figure 3.44A)

Red to pink to brown papules or nodules typically over scalp, around ears or forehead, ± grouped, ± painful

Histology: vascular proliferation with ‘hobnail’ endothelial cells (protrude into lumen), surrounding eosinophils

Treatment: surgical excision (1/3 recur)

Behçet’s disease
(Figure 3.44D–F)

Ulcerations; acneiform, papulopustular, EN-like or pseudofolliculitis-type lesions; ± arthritis, neurologic involvement, bowel aphthae, thrombophlebitis; HLA-B51

Histology: ulcerated epidermis or superficial pustule, diffuse neutrophilic infiltrate in dermis, ± lymphocytes, histiocytes, sometimes vasculitis

Treatment: colchicine, dapsone, thalidomide, TNF-inhibitors

{Diagnostic criteria: oral ulcers (at least three times in 12 month period) PLUS at least two of the following:
- recurrent genital ulcers - positive pathergy test - ocular (uveitis, retinal vasculitis) - skin findings (see above)}

Sweet’s syndrome
(Figure 3.45A)

Tender, erythematous edematous papules and plaques typically over face or upper extremities; ± fever, malaise, leukocytosis; vesicobullous variant frequently associated with myelogenous leukemia

Histology: variable epidermal changes, superficial dermal edema with diffuse neutrophilic infiltrate ± lymphocytes, histiocytes, eosinophils, no true vasculitis

Treatment: oral corticosteroid × 4–6 weeks, KI, dapsone, colchicine; 30% recurrence

{Diagnostic criteria: abrupt-onset of typical eruption + typical histopathological findings PLUS 2 of following:

1. preceded by associated infection/vaccination, drug exposure, current malignancy/inflammatory disorder or pregnancy
2. fever and constitutional symptoms 3. leukocytosis 4. excellent response to systemic corticosteroids}

Pyoderma gangrenosum
(Figure 3.45B, C)

{Vesicobullous type most common with acute or chronic myelogenous leukemia}

Tender papulopustule with violaceous induration → bulla or expanding ulcer w/ purulent base, undermined border; cribriform scar; pustular, bullous, granulomatous variants

{50% PG with systemic disease: IBD, arthritis or hematological disorders (if monoclonal gammopathy, usually IgA)}

Histology: early lesions with leukocytoclasia, neutrophilic infiltrate, advanced lesions with marked necrosis of tissue with infiltrate of mononuclear cells

Treatment: none with consistent efficacy; oral corticosteroid ± steroid-sparing agents