A. J. Kanwar

CUTANEOUS MASTOCYTOSIS IN CHILDREN: AN INDIAN EXPERIENCE

To the Editor: I read with great interest the article by Clifton et al (1) regarding immunotherapy of warts with mumps or Candida antigen. Cassone et al (2) used the term “microbial biological response modifier” to describe the immune modulatory effects of infectious microbes such as Candida albicans . They reported that Candida mannoprotein fragment F2 (MP-F2) is one of the strongest attractants of neutrophils. MP-F2, a powerful immunomodulatory fraction from the cell wall of C. albicans , is an active inducer of interleukin (IL)-8, tumor necrosis factor (TNF)α , IL-6, and IL-1 β production by human polymorphonuclear (PMN) cells and monocytes (2). Further, MacPhee et al (3) recovered CD4 + and CD8 +

Anhidrotic ectodermal dysplasia with palmoplantar keratoderma: an unusual presentation

Anhidrotic ectodermal dysplasia (AED), or Christ–Siemens–Touraine syndrome, was first described in 1848 by Thurnam. 1 It is characterized by a partial or complete absence of sweat glands, hypotrichosis and hypodontia. The mode of inheritance is predominantly X‐linked but an autosomal recessive pattern has also been observed. 2 Palmoplantar keratoderma is classically a component of hidrotic ectodermal dysplasia; 3 however, we report herein two brothers who had classical manifestations of AED along with palmoplantar keratoderma.

Sjögren-Larsson syndrome in an Indian child.

To the Editor: In the May/June 2003 issue, Pimentel et al (1) reported two children from Brazil with granular parakeratosis (GP), a rare dermatitis previously reported as occurring in the intertriginous areas of adults. Both children had been using Hipoglos pomade (Procter & Gamble do Brazil & Cia). The authors did not mention the fact that Hipoglos is a topical preparation consisting of vitamins A and D and zinc oxide. Granular parakeratosis has been reported in five other children less than 2 years of age. Each instance was associated with the topical application of zinc oxide paste (2) or Desitin (3), which contains zinc oxide. We recently evaluated an otherwise healthy 1-year-old boy with a 7-month history of pruritic, hyperkeratotic plaques in the groin bilaterally and around the penis. The patient had been treated with multiple topical preparations, including zinc oxide, pimecrolimus, tacrolimus, silver sulfadiazine, vinegar, nystatin, econazole, and triamcinolone. The differential diagnosis at presentation included irritant or contact dermatitis, psoriasis, dermatophyte infestation, acanthosis nigricans, and HaileyHailey disease. A specimen from a plaque showed the characteristic histopathologic changes of granular parakeratosis, including thick, compact basophilic parakeratosis without evidence of fungal elements with periodic acid-Schiff (PAS) staining. The boy’s mother was instructed to apply Vaseline and discontinue all other topical therapy, which resulted in gradual, complete resolution of the dermatitis without recurrence in 6 months of follow-up. Although an initial candidal or fungal infection cannot be entirely excluded, our patient’s presentation appears to be consistent with the previously reported conclusion that this is an unusual reaction to topical medicine and, perhaps more specifically, zinc oxide. One theory for the pathogenesis of GP involves a defect in the processing of profilaggrin to filaggrin, which maintains the keratohyaline granules in the stratum corneum (4,5). Jin et al (6) showed that zinc oxide applied topically to both incised and intact mouse skin significantly increased the mitotic index of the epidermal basal cells. The hyperkeratotic and acanthotic appearance of GP may be due in part to these same effects of topical zinc oxide on human skin. In addition to its proposed antimicrobial properties (7), increased proliferative activity of basal cells may be another reason for the enhanced wound healing seen with the topical application of zinc oxide pastes. Granular parakeratosis is likely an underreported reaction that should be considered in the differential diagnosis when children present with a hyperkeratotic dermatitis, particularly in the diaper area, where zinc oxide is often applied for various dermatoses.

ALOPECIA AREATA IN INFANTS: IS IT UNCOMMON?

To the Editor: Mycosis fungoides is a lymphoproliferative neoplasm, a T cell lymphoma. It is primarily a disease of adults but can start in the first decade of life as a hypopigmented patch. A paper on this disease, including different treatment modalities, was published in Pediatric Dermatology in 2000 (1). I would like to report a 9-year-old girl with hypopigmented mycosis fungoides successfully treated with UVA1-light (340–400 nm). We evaluated a 9-year-old, otherwise healthy white girl from Sweden with a solitary hypopigmented patch (5 × 9 cm) in her left groin (Fig. 1). The center of the patch was erythematous and scaly. It was first identified 4 years earlier and unsuccessfully treated with midpotency topical corticosteroid ointments. A biopsy specimen showed a bandlike infiltrate of atypical lymphocytes with cerebriform nuclei and extensive epidermotropism with the formation of Pautrier microabscesses. Immunohistochemical analysis revealed CD4+ and CD8+ atypical T lymphocytes. There was a monoclonal rearrangement of the T cell receptor gamma gene. Results of blood analyses were normal and no enlarged lymph glands were found. Results of chest radiographs and abdominal scan ruled out systemic involvement. The patient was treated with UVA1 irradiation. One month after the treatment was finished, the patch had decreased in size to a diameter of 7 cm. At this time, microscopy still showed a bandlike, lichenoid infiltration in the superficial part of the dermis. Atypical lymphocytes were also seen in the epidermis but no abscess formation. The T cell receptor gamma gene was now polyclonal. One year after treatment, the patient was in complete remission. Four years after therapy, the skin of the previously hypopigmented patch was normal. Psoralen + UVA-light (PUVA) is an effective treatment modality for mycosis fungoides in childhood (1). UVA1-light is a new treatment strategy for mycosis fungoides. This therapy resulted in complete remission including repigmentation for my patient. The girl is now free from disease 4 years after treatment. Recent in vitro studies have shown that UVA1-light induces immediate apoptotic cell death in human T lymphocytes (2). UVA1 generates reactive oxygen species (ROS), i.e., singlet oxygen or superoxide anions. ROS initiate lipid peroxidation of cytoplasmic membranes leading to structural and functional alterations of the cell, followed by apoptosis.