Chaganti Ratnakar

Disseminated Cutaneous Rhinosporidiosis

Two cases of longstanding rhinosporidiosis developed widespread asymptomatic nodular skin lesions. Cutaneous examination showed multiple, discrete, sessile and pedunculated, smooth and warty, friable nodular lesions of variable sizes and shapes. Histopathology of representative skin lesions showed hyperplastic epidermis with sporangia containing spores in the upper dermis diagnostic of rhinosporidiosis. Epidemiological data about rhinosporidiosis at Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India, is presented. Possible modes of dissemination to the skin and differential diagnosis are discussed in relation to this rare manifestation of rhinosporidiosis.

Primary Liposarcoma of the Cervix: A Case Report

A case of primary liposarcoma of the cervix is described in a 59‐year‐old postmenopausal female who was diagnosed clinically as having a cervical fibroid. Pathologic features and the probable histogenesis are discussed. Primary liposarcoma of the cervix does not appear to have been described in the literature.

Pebbling of the skin: a marker of Hunter's syndrome.

Abstract: A 9‐year‐old boy, the third child of nonconsanguinous parents, presented with asymptomatic, solid, raised skin lesions over the upper back. They first appeared at the age of 4 years. Gradually similar lesions appeared over the chest, neck, arms, and thighs. On examination, he had firm, hypopigmented to skin‐colored papules and nodules which coalesced to form ridges in a reticular pattern (pebbling of the skin) and were arranged bilaterally and symmetrically over the scapulae, pectoral region of the chest, and lateral aspects of the arms and thighs. They resembled sulci and gyri of the brain. He had normal intelligence, short stature, coarse facial features, thick lips, a large tongue, clear corneas, a protuberant abdomen with hepatosplenomegaly, and broad hands with clawlike contractures of the distal interphalangeal joints. Investigations revealed cardiomegaly and proximal tapering of metacarpal bones. Although peripheral blood smear and urine spot test for mucopolysaccharides were negative, histopathology of a representative skin lesion was compatible with the diagnosis of Hunters syndrome. The case is reported for its rarity and the typical skin lesions, the recognition of which may be helpful in diagnosis and genetic counseling.

Multiple Leiomyoma Cutis Associated with Becker's Nevus

To the Editor: The constituent epidermal, melanocytic, and pilar elements of Beckers nevus may demonstrate a spectrum of hamartomatous changes that may be congenital or acquired (1). We report a case of multiple leiomyoma cutis with mottled pigmentation and open comedones, probably representing the extreme form of Beckers nevus. Case Report: A 22-year-old male presented with a 10 year history of painful multiple swellings over the left side of the back. The number of lesions and pigmentation suddenly started increasing about 3 years previously. A 15 x 20 em area over the left side of the lumbar region crossing the midline of the back (Fig. 1) was involved with background freckle-like pigmentation and blackheads (open comedones). These were topped by 0.5 to 2 em sized, multiple, tender, skin-colored, soft to firm papular and nodular lesions. Histopathological examination of multiple sections of the representative skin lesion showed an ill-defined tumor with overlying epidermis (Fig. 2). The tumor was composed of interlacing bundles of smooth muscle fibers, which had eosinophilic cytoplasm and blunt-edged nuclei. Masson trichrome stain stained these muscle fibers densely red and the collagen blue. The epidermis showed a slight increase in the basal layer pigmentation. No nevus cells were seen in the dermis. The diagnosis of leiomyoma cutis with Beckers nevus was based on the above findings. Comments: The smooth muscle bundles in Beckers nevus, indistinguishable from those seen in smooth muscle hamartoma, have led to some controversy about the relationship of these two entities (2). Clinically, smooth muscle hamartoma presents as an asymptomatic single patch, most commonly in the lumbar region (3). It may present at birth or arise in childhood Or early adulthood (2, 4). Usually, there are small, follicular papules throughout the patch, 719

Cutaneous Horn: A Brief Review and Report of a Case

A case of extensive verrucae vulgaris with cutaneous horns in a 32‐year‐old man is reported. Accompanying terminal phalangeal bone absorptive changes in the fingers of both hands, resulting from subungual warty lesions, are highlighted. The existing literature on the subject of cutaneous horn is briefly reviewed.

Multiple keratoacanthoma with neonatal onset in a girl.

To the Editors: Lichen nitidus (LN) is an uncommon cutaneous chronic eruption that usually affects children and young adults. It was described by Pinkus in 1907 (1) and is characterized by the presence of 1 to 2 mm, asymptomatic, skin-colored papules with a flat, shiny surface. Cutaneous lesions of LN are most often localized to the upper extremities, penis, abdomen, chest, and buttocks, but in some cases they can be generalized (2–4). The association with other systemic diseases is extremely rare, and to our knowledge LN has never been described in association with juvenile chronic arthritis. We report a 3-year-old girl who suffered from generalized lichen nitidus and developed polyarticular juvenile chronic arthritis months later. At the age of 11 months the infant began to have a generalized eruption consisting of papular lesions which were diagnosed by biopsy as lichen nitidus. At the age of 15 months, 10 days after receiving a triple viral vaccine, she developed a high fever with swelling and pain in the wrists and ankles for 4 days. When she was 24 months old she had a similar episode without previous infectious or vaccination antecedent that recurred at the age of 28 months, 3 days after she received antimeningococcal A+C vaccine. In spite of clinical improvement observed after these episodes, the swelling and impaired function of the affected joints persisted. Physical examination showed numerous small, flesh-colored papules which were generalized but predominated on the right side of the body, symmetrical polyarthritis of the proximal interphalangeal joints from the second to the fourth, metacarpusphalangical from the first to the fifth, wrists, knees, and ankles. Laboratory findings including complete serologic studies were normal. Rheumatoid factor and antinuclear antibodies were negative. Histologic examination of a skin biopsy specimen revealed a circumscribed lymphohistiocytic infiltrate in the superficial dermis that was characteristic of LN. After treatment with ibuprofen, methotrexate, and prednisone, a good clinical evolution was observed, with an increase in physical activity and improvement of articular function. Most patients suffering from LN have a spontaneous resolution of the lesions after several years, so treatment is not required. Some authors recommend treatment with topical steroids, astemizole, systemic steroids, or PUVA in generalized LN (4,5). Etretinate and acitretin have also been used in palmoplantar LN (6). In our patient, apart from hydration of the skin, specific treatment was not used, the lesions resolving with a residual hypopigmentation 2 years after onset. Familial cases or association with systemic diseases, aside from Crohn disease and atopic dermatitis, have not been reported (7,8). Kano et al. (7) described a 22-yearold man diagnosed with Crohn disease and erythema nodosum who developed LP and LN 2 years later. The clinical course of his LN and LP was independent of intestinal and erythema nodosum lesions. After evaluating immunohistochemical and gene expression studies, these authors suggest that the lesions of LN and LP are likely to be mediated by T cells with antigen specificity distinct from those that cause erythema nodosum and intestinal lesions. The observed association of generalized LN with juvenile chronic arthritis in our patient is the first description in the literature to our knowledge. In spite of the frequency of cutaneous manifestations in the connective tissue diseases, they are not as evident as in juvenile chronic arthritis, excepting the characteristic exanthem of this disease. Recently the development of skin alterations such as a rash in patients being treated with Dpenicillamine and gold salts has been reported (9). Two women suffering from rheumatoid arthritis who developed lesions of LP while being treated with sulfasalazine were described by Kaplan et al. (10). In our patient, the appearance of LN without administration of some type of antirheumatoid drug only a few months before the development of juvenile chronic arthritis, the coexistence of the lesions, and the occurrence of new outbreaks of LN, coinciding with worsening of the arthritis suggest to us that both diseases could be due to the same mechanism, probably immune. However, additional clinical observations and more complete studies are necessary to establish the possible link between these processes.

Pyoderma Gangrenosum in Childhood: Case Report

Two cases of pyoderma gangrenosum occurring in children after an exanthem are reported because of rarity and clinical interest.

A Rare Association of Localized Gigantism with Tuberous Sclerosis

An unusual association of localized gigantism with hypertrophy of the long bones and soft tissues in the left lower limb in an 18‐year‐old male with tuberous sclerosis (TS) is reported. The significance of this association is discussed from the point of view of its common neural crest origin during embryogenesis.

ASCHER'S SYNDROME: AN UNUSUAL CASE WITH ENTROPION

A 26-year-old unmarried woman presented with the complaints of photophobia, watering and difficulty in opening the eyes fully, along with wrinkling of the skin over all four eyelids. These had developed progressively over the previous 2 years following recurrent periodic episodes of periorbital edema, which recurred a week prior to her menstrual periods, with mild itching over the eyelids. She had not had any form of topical treatment and had only been treated with antihistamines systemically, to no relief. No other family member had similar complaints, and the patient gave no past history of symptoms suggestive of atopy.

Lymphangioma circumscriptum of the skin and tongue associated with cystic hygroma.

A 25 year-old-woman with lymphangioma circumscriptum of the skin and tongue associated with cystic hygroma is reported for its rarity and unique presentation.