Combined carvedilol and gabapentin treatment induces a rapid response in red scrotum syndrome.

Red scrotum syndrome (RSS) is a rare dermatologic condition characterized by persistent erythema and analgesia of the male genitalia that cannot be attributed to contact or atopic dermatitis or acute or chronic infections. Treatment of RSS is challenging since it often fails to respond to corticosteroids, antifungals, antivirals, and antibiotics. Several reports described RSS patients who responded to gabapentin, pregabalin, and ?-adrenergic receptor blockers, suggesting a neuropathic etiology. Here we present a refractory RSS case with rapid clinical improvement on a combined carvedilol plus gabapentin therapy. We suggest that RSS manifestations are driven by neurogenic inflammation and that the efficacy of gabapentin/carvedilol relates to the suppression of the neuro-immuno-epidermal axis.

Trichomegaly in a 3-year-old girl with alopecia areata.

We report here a case of bilateral trichomegaly associated with alopecia areata in a 3-year-old girl, healthy except for mild atopic dermatitis. Trichomegaly is a rare condition and, in many cases, is a side effect of medication such as ophthalmic solution prostaglandin analogs and epidermal growth factor receptor inhibitors. Trichomegaly has also been associated with acquired medical conditions such as HIV, systemic lupus erythematosus, anorexia nervosa, porphyria cutanea tarda, hypothyroidism, and dermatomyositis. In very rare circumstances, trichomegaly has been described as part of congenital conditions such as Oliver-McFarlane syndrome. We believe that the development of bilateral trichomegaly in conjunction with alopecia areata in this patient represents a novel finding as it occurred in the absence of any significant health problems, congenital abnormalities, or medications.

Systemic mastocytosis: classification, pathogenesis, diagnosis, and treatment.

Mastocytosis is a heterogeneous entity that may present as either a cutaneous or systemic disease. Progression of pediatric cutaneous mastocytosis (CM) is uncommon, but in adults, this condition persists and often progresses to systemic disease. Mast cell proliferation and differentiation from stem cell precursors depend on a number of factors, including a mast cell tyrosine kinase receptor (kit) and its ligand (the stromal cell-derived cytokine stem cell factor). A gain-of-function mutation in codon 816 of c-kit is frequently present in mast cells of patients with systemic mastocytosis (SM). The diagnostic approach for a patient with suspected mast cell disease includes a thorough skin examination, a skin biopsy, a serum tryptase level, and bone marrow aspiration and biopsy. The treatment is directed toward avoidance of triggers of mast cell mediator release and management of symptoms. Aggressive cases are managed with cytoreductive therapies, such as interferon alfa-2b and cladribine. Research has been directed at more specific treatment modalities, including specific kit tyrosine kinase inhibitors.