Anaplastic lymphoma kinase-positive primary cutaneous anaplastic large cell lymphoma- Is it a new variant.

Anaplastic large cell cutaneous lymphomas are clinically and pathologically heterogeneous, CD30 + (Ki-1) lymphoproliferative disorders. The importance of anaplastic lymphoma kinase (ALK) positivity is well known in the prognosis of primary systemic anaplastic large cell cutaneous lymphomas; however, the same in primary cutaneous anaplastic large cell cutaneous lymphomas is not much clear. Herein we report a 65-year-old male with an 18-month history of minimally pruritic localized nodulo-plaque lesion over lower back. Histology revealed cutaneous large cell lymphoma and immunohistochemical staining showed positivity for CD30, CD3 and ALK. The role of ALK positivity in pcALCL is discussed in this article.

Keratosis follicularis spinulosa decalvans in a female.

Keratosis follicularis spinulosa decalvans (KFSD), is a rare follicular syndrome associated with widespread keratosis pilaris and progressive scarring alopecia. This genodermatoses often starts at infancy or early childhood with an X-linked mode of inheritance. Males are predominantly affected and females frequently show no disease or only a mild form. We describe this not so common entity of KFSD in a nine year old female child.

Paraneoplastic multicentric reticulohistiocytosis: A clinicopathologic challenge.

Multicentric Reticulohistiocytosis (MRH) is a rare, systemic non-Langerhans cell histiocytosis (non-LCH) with prominent joint and skin manifestations. It is mostly self limiting. However, 15-30% of the cases are associated with malignancy and carry a poor prognosis. We report the case of a 42-year-old man who presented with multiple reddish-brown papules that on biopsy showed aggregates of oncocytic histiocytes with several multinucleate giant cells. Immunostains were positive for CD 68, CD 45 and were negative for S-100, CD1a. An impression of multicentric reticulohistiocytosis (MRH) was made, with the recommendation to screen for malignancy. Electron microscopy of the skin lesions showed features consistent with non-Langerhans cell histiocytosis. The patient was later diagnosed with acute myeloid leukemia at a follow-up visit several months later. Thus, it appears prudent to screen and follow-up adults with MRH, to identify an underlying malignant condition.

Berardinelli-Seip syndrome in a 6-year-old boy.

A 6-year-old boy presented with abnormal habitus since birth, delayed language development, history of frequent falls since 9 months, and fever since 1 week. He was found to have hyperandrogenic features, generalized paucity of fat, generalized muscular overdevelopment, and brownish pigmentation over the flexural creases. Skin biopsy demonstrated features suggestive of acanthosis nigricans with an absence of subcutaneous tissue. After further investigation, a diagnosis of Berardinelli-Seip syndrome with bilateral pneumonia and generalized tonic clonic seizures was made. Clinical features, histopathology, differential diagnosis, and prognosis of this rare disorder have been discussed.

Familial dyskeratotic comedones.

Familial dyskeratotic comedones (FDC) is a rare autosomal dominant inherited condition, characterized by widespread, symmetrically scattered, comedone-like, hyperkeratotic papules, which are cosmetically unappealing. These lesions appear around puberty and show a predilection to involve the trunk, arms and face. The lesions are asymptomatic and gradually worsen with time. Histology shows invagination of the epidermis with a lamellar keratinous plug and focal evidence of dyskeratosis. This condition is generally refractory to therapy. We report here two cases with this rare disorder who had a strong familial history of the same disorder.

Fine needle aspiration cytology of trichofolliculoma: a case report.

The cytomorphology of skin adnexal tumours has not been described extensively in literature. We report the first case of trichofolliculoma, a hair follicle hamartoma, which was subsequently diagnosed by excision biopsy. A 19 year old woman presented with a nodule on the dorsal aspect of the finger which was clinically suspected to be a myxoid cyst. FNA showed several cohesive as well as arborising branched groups of squamous cells with evidence of keratinisation. The background had dispersed and loosely clustered sebaceous cells. The excision biopsy revealed a trichofolliculoma. Cytology is a useful tool in the diagnosis of skin adnexal tumours. The finding of cohesive, branching keratinized squamous cell clusters admixed with sebaceous cells may suggest a diagnosis of trichofolliculoma, as was seen in the present case.

Cutaneous vasculitis--a dynamic process posing diagnostic challenge.

AIM: To characterize the clinicopathologic features and to assess the therapeutic outcome in cutaneous vasculitis. MATERIAL AND METHODS: Fifty biopsy proven cases of cutaneous vasculitis seen between January 1998 and July 1999 were studied. RESULTS: The commonest presentation was palpable purpura. The site most commonly affected was the extremity, irrespective of the age (adults - 40 and children - 10) and sex. The histopathologic picture ranged from an acute to chronic process, which besides the classic picture included bullous presentation, granulomatous histology and nonspecific features. Clinical correlation and investigations including direct immunofluorescence (DIF) were required to differentiate primary from secondary vasculitis (SLE-4, dermatomyositis-2, rheumatoid artritis-1, HIV-1, septicaemia-1 and drug reaction 2). DIF was diagnostic in 13 out of 21 cases providing evidence of an immune-mediated pathogenesis. Drugs used in the treatment included dapsone, colchicine, pentoxyphiline and steroids. CONCLUSIONS: The clinical picture and outcome of primary cutaneous vasculitis were benign while the prognosis of secondary vasculitis depended on the primary disease, irrespective of the histopathological picture.

Eczema herpeticum in two elderly patients.

Two elderly patients with eczema herpeticum are being reported. The First patient did not respond to the conventional dose of acyclovir. The second case was seen in a patient with air borne contact dermatitis.

Malignant pheochromocytoma with cutaneous metastases presenting with hemolytic anemia and pyrexia of unknown origin.

We describe a 43 year old female who presented with pyrexia of unknown origin associated with Coomb's negative hemolytic anemia and impaired liver function tests of six months duration. A routine abdominal computerised tomographic scan showed a mass in the left adrenal which was excised at laparotomy and histologically diagnosed as pheochromocytoma. The hemolysis continued to worsen with development of resistance to steroid therapy. Subsequently she developed multiple firm to hard painless cutaneous nodules which were aspirated for cytologic examination. The cytology picture was that of a neuroendocrine tumour with cell morphology similar to that of the adrenal pheochromocytoma. Such an association of hemolytic anemia, pyrexia and cutaneous metastases in a case of malignant pheochromocytoma has not been described earlier in the medical literature.