Pediatric Dermatology Vol. 14 No. 3 204-208, 1997 Granulomatous Slack Skin in Childhood Francisco M. Camacho,* Giinter Burg,t Jose C. Moreno,* Ricardo G. Cainpora,^ Jose L. Villar? ^Department of Medical-Surgical Dermatology, Hospital Universitario Virgen Macarena. Universidad de Sevilla. fDepartment of Dermatology, University Hospital, Ziirich, Switzerland, and tDepartment of Pathology, Universitario Virgen Macarena, Universidad de Sevilla Abstract: Granulomatous slack skin is an uncommon cutaneous T- helper cell lymphoma closely related to mycosis fungoides. To the best of our knowledge this disease has not been previously described in chil- dren. We report on an 11-year-old boy who presented with painless slack skin masses in the neck, right axilla and arm, anterior wall of the abdo- men, both inguinal regions, and the malleolar and dorsal aspects of the feet. The disease started 3 years earlier with erythematous lesions on the neck and wrists. Histologic examination of a specimen from the abdomi- nal mass revealed an extensive lymphoid Infiltrate with scattered multi- nucleated giant cells extending from the papillary dermis to the subcutis. The lymphoid cells showed the following immunophenotype: CD43-f (MT1), CD45+, CD45RO't-, CD20-. The phenotype of the gianf cells was lysozyme positive, 0068+ and Mac387-. The tumoral lymphoid cells had clonal rearrangement for the gene of the beta chain of the T-cell receptor (CpTCR). The disease could be controlled with systemic glucocorticoids. Due to the presence of many histiocytes arranged in aggregates in the papillary and mid-dermis, this case was initially considered to be a cuta- neous form of histiocytosis. We recommend deep and extensive biopsies in patients with slack skin disease. Granulomatous slack skin (GSS) disease is an unusual cutaneous lymphoma that is closely related to mycosis fungoides. It was described originally by Convil el al. (1) as a progressive chronic atrophic granulomatous dermo- hypodermitis of unknown cause. GSS currently is con- sidered to be a cutaneous lymphoma ofthe T-belper cells that has a progressive course, sometimes marked by transformation to disseminated lymphoma ( 2 ^ ) . None of the 20 cases published in the literature (5,6) involve.s a pediatric patient. We describe the clinical, histopatho- logic, immunohistochemical, and genotypic findings in a Addres.? correspondence lo Francisco M. Camacho. Department of Medical-Surgical Dermatology. Hospital Universitario Virgen Macarena. Avda.Dr.Fedriani 3. 41009 Sevilla (Spain). case of GSS initially simulating "pure cutaneous histio- cytosis X" (7) in a child. CASE REPORT In September 1990 an 11-year-old boy was referred to us from another hospital with soft, painless skin masses in his neck, right axilla and arm, anterior wall of the abdo- men, both inguinal regions (Fig. 1), and the malleolar and dorsal regions of both feet. The disease had begun 3 years earlier as erj'thematous lesions of the neck and 204