Clinicopathological and Immunological Profile of Patients with Cutaneous Manifestations and their Relationship with Organ Involvement in Systemic Lupus Erythematosus Attending a Tertiary Care Center of Eastern India.

BACKGROUND: Lupus erythematosus (LE) is an autoimmune disorder with diverse clinical manifestations ranging from mild cutaneous disorder to life-threatening systemic illness and associated with varying immunological parameters. AIM: We conducted a study in a tertiary care center of eastern India to determine the clinical pattern, immunological profile of patients with cutaneous manifestations of systemic LE (SLE) and their relationship with organ involvement. MATERIALS AND METHODS: Fifty-five consecutive patients attending dermatology OPD having features consistent with cutaneous LE and fulfilling the criteria of SLE were included. After proper history taking and clinical examination, routine blood and antinuclear antibody (ANA) profile, histopathological examination, and direct immunofluorescence test were undertaken. RESULTS: Among 55 patients, 49 were female. ANA positivity was the most common association, followed by photosensitivity, malar rash, arthritis, oral ulcer, immunological markers, renal system involvement, discoid rash, serositis, central nervous system (CNS) involvement, and least common being the hematological involvement. Vacuolar basal cell degeneration was the commonest epidermal change and upper dermal periappendageal and perivascular lymphocytic infiltration was the commonest dermal change observed on histopathological examination. On direct immunofluorescence (DIF) granular pattern was seen in majority of patients. Statistically significant risk of kidney involvement was present both when patient had bullous lesions and DIF positivity of unexposed (DIF-UE) skin. CNS involvement was seen in five patients and it was found to be significantly associated with purpuric lesions. CONCLUSION: This study reveals cutaneous lesions and DIF testing could be reliable predictors of systemic involvement and strongly suggests DIF testing, routinely in all patients of SLE.

Darier's Disease in Gastric Malignancy: An Unusual Paraneoplastic Phenomenon.

Darier's disease is an autosomal dominant genodermatosis resulting from ATP2A2 gene mutation. A 62-year-old male presented at our outpatient (OPD) with sudden-onset numerous dirty, warty papules over the head, neck, and back since 2 months. Histopathology of the skin lesions revealed acantholytic dyskeratosis suggestive of Darier's disease. He was referred to the gastroenterology department for some gastrointestinal (GI) symptoms where he was diagnosed with adenocarcinoma of the stomach and was subsequently operated. On his next visit to our department for follow-up, we found a marked diminution of the skin lesions in the absence of any specific treatment. In view of the above finding, we concluded that paraneoplastic dermatosis in the form of Darier's disease occurred in this patient. Paraneoplastic Darier's disease with gastric adenocarcinoma is not yet described in dermatology literature and is hence reported here.

Isolated tenosynovitis as a sole manifestation: the great mimicker still continues to surprise us.

A middle aged male presented with non-tender cystic swelling over left distal forearm since 1 year. No other cutaneous abnormality could be found except mild paresthesia of the overlying skin and equivocal thickening of the ipsilateral ulnar nerve. Routine investigation was within normal limits. Detailed workup of the patient including MRI of the lesion suggested the diagnosis as tenosynovitis with a soft tissue mass. Fine needle aspiration cytology from the cyst showed foamy macrophages and acid fast bacilli; while PCR of the aspirate confirmed the etiological agent as M. leprae. We, thus, report a unique case of isolated tenosynovitis as a sole manifestation of pure neural leprosy which is extremely rare in world literature.

A curious case of sweating blood.

Hematohidrosis is a very rare condition in which an individual sweats blood. It may occur in an individual who is suffering from extreme levels of stress. Various causative factors have been suggested like component of systemic disease, vicarious menstruation, excessive exertion, psychogenic, and unknown causes. Fear and intense mental contemplation are the most frequent causes. It may also occur in bleeding disorders. We here report a case where bloody sweat was discharged from the forehead, face, and body episodically in a 12-year-old healthy girl with no bleeding disorder or any other underlying cause. All investigations done were within normal limits, except low intelligent quotient and loss of insight. The patient was given atropine sulphate transdermal patch with marked improvement in severity.

Ichthyosiform large plaque parapsoriasis: report of a rare entity.

Large plaque parapsoriasis (LPP) is an idiopathic, chronic scaly dermatosis classified within parapsoriasis group of diseases, occurring commonly in middle aged patients of all races and geographic regions. LPP and its variants are closely related to the patch stage of mycosis fungoides. The two types of LPP mostly described are the poikilodermatous and retiform parapsoriasis. We are reporting an ichthyosiform LPP for its rarity.