I have long had the idea that God created psoriasis and warts to teach dermatologists the merits of humility.
(Vincent Joseph Derbes, 1981)
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Monday, October 10, 2011
Cutis laxa acquisita associated with cutaneous mastocytosis
Vikram K. Mahajan MD,
Nand Lal Sharma MD,
Geeta Garg MD
Article first published online: 28 JUL 2006
A female child, a product of a nonconsanguineous parentage and normal pregnancy, was seen first at the age of 4 years. She had suffered from recurrent episodes of multiple, erythematous, confluent plaques and a few bullous lesions all over the body since infancy. The child was irritable but had no history of flushing, dyspnea, wheezing, or frequent diarrhea. Darier's sign was strongly positive and systemic examination did not reveal any abnormality. Routine laboratory investigations were normal and a skin biopsy specimen revealed an unremarkable epidermis and dense proliferation of round to spindle-shaped mast cells which stained positive for metachromatic granules (Fig. 1). With the diagnosis of urticaria pigmentosa, she was managed with antihistaminic drugs and the parents were advised to handle the child gently. She did not report for further follow-up.
Recently, the child, now 8 years of age, was brought again with finely wrinkled, lax, and redundant skin, especially of the face, giving her a senile appearance (Fig. 2). Systemic examination did not show any abnormality. History revealed that the child had been asymptomatic during the last 4 years even without treatment, but developed slowly progressive lax and pendulous skin. A repeat biopsy from the lax facial skin revealed sparse and fragmented elastic fibres, normal collagen, and a perivascular and intradermal cellular infiltrate composed of metachromatic mast cells. Her serum α1-antitrypsin levels were 190.0 mg/dL (normal, 93–224 mg/dL) and a pulmonary function test showed no abnormality. Treatment with antihistaminic drugs was started again and she was advised to have a cosmetic facial uplift at a later stage.
Figure 2. Patient with urticaria pigmentosa seen at 8 years of age showing cutis laxa and a senile appearance
*.General Surgeon (Hue,VN); *. ENT (Hue,VN); *. Leprosy Surgeon (I.L.A.D); *.Leprologist (C.E.S de Léprologie, Dakar, Senegal); *.Level II Dermatologist (HCMC);
*.Speaker at The Combined Congress of 27th Asean Orthopedic Association-6th Vietnamese Orthopedic Association-12th Spine society of HCM city (HCMC-December, 2007); *.International Hospital Federation 1996 Associate Fellow (Hongkong-London);
*.Fellowship in Saint- Louis Hospital at Department of Plastic Surgery and Department of Dermatology (Paris, 2008).
*.Fellow of Ho Chi Minh Society for Laser in Medicine and Surgery ;
*.Fellow of Vietnamese Society of Dermatology and Venereology;
*.International Fellow of American Academy of Dermatology