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Diffuse cutaneous mastocytosis responding to cyproheptadine
U Enomoto1, H Kusakabe, T Matsumura
1Department of Dermatology, Osaka Medical College, Osaka, Japan.
Insights
A rare infant skin condition presents with leathery skin and blisters. Cyproheptadine, an anti-serotonin drug, effectively reduced blistering in a case of congenital bullous dermatosis.
Area of Science:
- Pediatric Dermatology
- Dermatopathology
- Mast Cell Biology
Background:
- Congenital bullous dermatoses represent a group of rare blistering disorders presenting at birth.
- Mastocytosis is characterized by abnormal mast cell proliferation, which can manifest in the skin.
Observation:
- A 3-month-old infant presented with generalized, thickened, leathery skin and recurrent blisters since birth.
- Blisters healed without scarring, and Darier's sign was positive.
- Microscopic examination revealed a dense dermal infiltrate with mast cells and subepidermal bulla formation.
Findings:
- The infant showed no signs of systemic mastocytosis.
- Treatment with cyproheptadine, an antihistamine with anti-serotonin activity, significantly reduced blistering.
- Histopathology confirmed features suggestive of a bullous dermatosis with mast cell infiltration.
Implications:
- This case highlights a potential therapeutic option for congenital bullous dermatoses.
- Cyproheptadine's efficacy suggests a role for serotonin pathways in the pathogenesis of these conditions.
- Further research is warranted to explore anti-serotonergic agents in managing similar pediatric skin disorders.
Abstract:
A 3-month-old male infant was referred to our department with a generalized brown, thickened leathery skin and blisters which had been present since birth; the blisters developed recurrently at sites of minor trauma, healing without scar formation, although Darier's sign was positive. Microscopically, biopsy specimens showed a dense dermal band-like infiltrate with mast cells, whereas a biopsy from a vesicle showed subepidermal bulla formation. Physical examination did not reveal any systemic involvement with mastocytosis and the patient was treated with cyproheptadine, which has considerable anti-serotonin activity and, interestingly, reduced the degree of blistering; this treatment might therefore be tried in other cases.