Diffuse cutaneous mastocytosis masquerading as epidermolysis bullosa.

A 10-month-old boy presented with a history of a generalized cutaneous bullous eruption since 3 months of age. Emesis, flush, pruritus, and fatigue had accompanied relapsing episodes of sometimes extensive blistering. Histopathology showed dense dermal infiltrates of mast cells on hematoxylin and eosin and corroborating immunohistochemical staining. Laboratory examination revealed a markedly high level of serum tryptase. Based on these results and after consecutive staging, the patient was diagnosed with diffuse cutaneous bullous mastocytosis (BM). Mutation analysis detected a deletion mutation (del419) in C-Kit by direct exon sequencing. This rare entity must be considered in the differential diagnosis whenever a child presents with bullae and erosions. A crucial diagnostic hint is that rubbing of affected skin areas results in whealing (Darier's sign). A comprehensive diagnostic approach, advanced therapeutic strategies, regular follow-ups, and instruction of patients and relatives on prevention and prophylaxis are highly indicated.

Donor-site morbidity of the transverse musculocutaneous gracilis flap in autologous breast reconstruction: short-term and long-term results.

BACKGROUND: The authors have used the transverse musculocutaneous gracilis flap technique for autologous breast reconstruction after skin-sparing mastectomy since August of 2002. The donor site is closed in the manner of a medial thigh lift. The authors examined the short-term and long-term results of donor-site morbidity in their first 22 patients. METHODS: Nineteen patients underwent unilateral and three patients received bilateral breast reconstruction with a transverse musculocutaneous gracilis flap after skin-sparing mastectomy. Using a questionnaire, patients were asked about complaints resulting from elevation of the gracilis muscle and their satisfaction with the result, general condition, and sexuality. Cosmetic evaluations of the thigh donor site were performed independently by two plastic surgeons. RESULTS: To evaluate short-term results, mean follow-up of the 22 patients was 10 months. All patients were satisfied with the scar in the inguinal region. Concerning thigh symmetry, 42 percent of patients showed excellent results, 40 percent had good results, and 18 percent had fair results. With regard to the scars, 24 percent of patients had excellent results, 46 percent had good results, and 30 percent had fair results. Thigh shape was evaluated as excellent by 26 percent, good by 52 percent, and fair by 22 percent. Patients who had a unilateral gracilis donor site had a difference in maximal thigh circumference of 2.368 cm. Four years postoperatively, all patients would choose this kind of operation again. CONCLUSIONS: The medial thigh region allows the removal of a moderate amount of tissue, even in thin patients, with a very inconspicuous scar. The transverse musculocutaneous gracilis flap is safe for immediate reconstruction of small and medium-sized breasts, with minimal functional donor-site morbidity. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.