Macrodystrophia lipomatosa: Review of clinico-radio-histopathological features.

We report clinical, radiological, and pathological features of three cases of macrodystrophia lipomatosa, which is characterized by progressive proliferation of all mesenchymal elements, with a disproportionate increase in fibroadipose tissue. The peculiarity in our report is the involvement of contralateral hand and feet in our 1(st) case in contrast to usual presentations of this rare condition, which is often unilateral. In our 2(nd) case, the lateral aspect of foot was involved as against the usual finding of involvement of medial aspect in lower limbs, also another surprising finding in this case is increase in the size of lesion after puberty. Coming to our 3(rd) case, enlargement of almost whole of an upper extremity with profound involvement of middle, ring and little finger along with total sparing of the thumb is in itself an extremely rare occurrence as in upper limb, mainly the lateral aspect of hand and related digits bear the burnt and more over involvement of whole limb is itself an unique phenomenon.

Recurrent Pyogenic Granulomas with Underlying Arteriovenous Malformation: An Exclusively Rare Entity.

We report a case of recurrent localized pyogenic granulomas with underlying arteriovenous malformation in an 11-year-old male child on upper back with onset in early infancy.

Zinc-responsive acral hyperkeratotic dermatosis-A novel entity or a subset of some well-known dermatosis?

BACKGROUND: We are reporting a series of interesting cases, which presented to us with psoriasiform lesions distributed over the acral regions of the body. The cases are unusual because they were resistant to conventional treatment modalities like topical corticosteroids, tacrolimus and oral methotrexate but showed significant improvement on oral zinc therapy. MATERIALS AND METHODS: Ten patients with characteristic clinical features of distinctive hyperkeratotic plaque in the acral areas, who were resistant to treatment by different modalities including potent topical steroids and oral methotrexate, were included for detailed investigations. A proper history was taken and relevant laboratory investigations were done which included blood count, urine, liver function, renal function, hepatitis-C virus serology and serum zinc levels. Patients were followed up every 2 weeks. Histopathological examinations of the lesional tissue were done at baseline and after 6 weeks of therapy. Patients were given oral zinc daily and no other treatment during the 6 weeks course. RESULTS: All our patients were non-reactive to hepatitis-C. Of the ten patients only one patient (10%) showed low titer of serum zinc, another (10%) showed higher zinc level, while the rest of the patients had normal zinc level. Five of our patients had chronic renal failure, one had Grave's disease and the remaining had no associated systemic illness. Histopathology mostly showed hyperkeratosis, acanthosis, prominent granular layer, spongiosis and dermal infiltrate. After 6 weeks of follow up, all patients showed rapid and remarkable therapeutic response with zinc. CONCLUSIONS: We here report a series of patients, discernible because of their uniform clinical presentation of acral hypekeratotic plaques and in showing a noticeable response to zinc. Clinical, histopathological and laboratory investigations were done to rule out diseases of similar morphology including psoriasis, acral necrolytic erythema and lichen simplex chronicus. Authors understand that further studies with greater number cases and more detailed investigations are required to establish exact etio-pathogenesis and nomenclature of this distinct subset of patients.

Phakomatosis pigmentovascularis presenting with sturge-weber syndrome and klippel-trenaunay syndrome.

Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi. Four types and two subtypes have been described where subtype 'a' present only with cutaneous form and subtype 'b' also with systemic association like in Sturge-Weber syndrome or Klippel-Trenaunay syndrome. Hereby, we report a case where our patient presented with port-wine stain, Nevus of Ota, Sturge-Weber syndrome, and Klippel-Trenaunay syndrome; which has made it a rare combination.

Cutaneous manifestations of mixed connective tissue disease: study from a tertiary care hospital in eastern India.

CONTEXT: Mixed connective tissue disorder is an uncommon disease. Some scientists are reluctant to recognize it as a separate entity. Some others have defined this ailment. Cutaneous features of this condition are unique. Researchers from India have described these features to relate to those described in the studies from other parts of the globe. AIMS: This study aims to delineate the skin manifestations of clearly defined mixed connective tissue disease (MCTD) patients, to compare them with those established as overlap syndrome, and to relate them with studies from other parts of the globe. SETTINGS AND DESIGN: Successive patients who fulfilled the specific criteria for MCTD presenting in the skin outpatient department of a tertiary care hospital in eastern India were clinically examined from 2009 for 3 years. MATERIALS AND METHODS: The number of participants was 23 and the dermatological features of these were compared with 22 patients with overlap syndrome. The antibody to uridine-rich U1 ribonucleoprotein was measured for all patients. STATISTICAL ANALYSIS USED: SPSS (Version 17) and MedCalc (Version 11.6). RESULTS: THE MALE: Female ratio among the MCTD patients was 1:6.67 and that of the overlap syndrome was 1:10. Twenty patients of the MCTD group presented with synovitis as against only seven in the overlap group. Raynaud's phenomenon was present in some of the subjects. Puffy fingers were rare in our study. Facial numbness was reported by four of those suffering from MCTD. Antinuclear antibody (ANA) was essentially of a speckled pattern in this disease. CONCLUSIONS: Cutaneous indicators of MCTD are distinct from overlap syndrome. Knowledge of these manifestations prevalent in a region may lead to early diagnosis of the disease.

Zosteriform palisaded encapsulated neuroma: an unusual presentation.

Palisaded encapsulated neuroma (PEN) is an infrequent benign cutaneous neural tumor, which usually presents as solitary, asymptomatic, papule or nodule, often localized on the faces, around the orifices, in middle age with no sex preponderance. Rarely, it can be multiple. Both solitary and multiple lesions are not associated with neurofibromatosis or multiple endocrine neoplasica syndrome type 2B (MEN 2B). We hereby report, a rare case of multiple cutaneous PEN in a 30-year-old female who presented with multiple asymptomatic soft-to-firm papules and nodules in a dermatomal distribution on the face along the supply of the ophthalmic division of the trigeminal nerve with no signs of neurofibromatosis or MEN 2B.

Diogenes syndrome: a case report.

Cessation of normal skin cleansing seen in geriatric or self-neglected patients can cause accumulation of keratinous crusts on the skin. In the extreme end of this spectrum is a condition known as Diogenes syndrome (DS). These patients may have psychiatric disorders like paranoid disorders, mood affection, or temporofrontal dementia. Subjects are mainly the elderly but few cases in younger age group of patients have also been reported. Lesions of DS are usually found over upper central chest, back, and groin. In the young, lesions are mainly found over scalp, face, or arms. Absence of normal skin cleaning causes keratin and dirty debris to accumulate and with time form a thick shell. These debris can be secondarily infected by bacteria, fungus, and so forth. These skin lesions are not usually seen in individual with proper hygiene. We report a case of Diogenes syndrome in a 34-year-old young male patient who had associated schizophrenia.