Acne fulminans in a young man with granulomatosis with polyangiitis (Wegener's granulomatosis): A chance association or marker of serious systemic disease?

A 19-year-old man with granulomatosis with polyangiitis (Wegener's disease) presented with hemorrhagic facial nodules mimicking severe inflammatory acne (acne fulminans) as one of the first symptoms of the disease. The lesions were earlier treated as nodulocystic acne with isotretinoin without any benefit. Complete resolution was seen with pulsed methylprednisolone and oral prednisolone and mycophenolate mofetil thereafter. He also developed acute onset of severe pustular eruption of the face and a destructive ulcer of the auricle on two separate occasions. Facial lesions mimicking severe inflammatory acne, not responsive to standard treatment, may be a marker for more severe systemic disease such as Wegener's disease/granulomatosis with polyangiitis.

The story of a deformed leprous foot.

Eccrine syringofibroadenoma (ESFA) is a rare adnexal tumour of eccrine ductal proliferation. A 50 year old treated case of leprosy presented with a chronic non healing ulcer of 5 years duration on the deformity laden right foot. Multiple verrucous papules and plaques were seen surrounding the ulcer which showed histopathological findings consistent with ESFA. Although ESFA constitutes a rare association with leprosy, considering the load of treated cases in our country and elsewhere, it may represent an under-reported entity which requires more attention in the post elimination era.

A new occlusive patch test system comparable to IQ and Finn chambers.

BACKGROUND: A good patch test system should have good adhesion and contact, and minimal leakage; Finn and IQ patch test system have these properties but are expensive. AIMS: To develop a new cost-effective occlusive patch test system that had good contact with the skin and was non-irritant. METHODS: The system (designated Chamber X) was fabricated using a semi-permeable tape and a flexible virgin plastic chamber. Chamber X was developed by (i) selecting adhesive tape based on its non irritancy and adhesive potential (ii) testing plastic chamber material for its skin irritancy (iii) testing the assembled system against Finn, IQ and locally available chambers for irritancy, contact, leakage and occlusivity. RESULTS: Chamber X showed better occlusion than IQ, Finn and locally available chambers and was comparable to, (P > 0.05) IQ and Finn in terms of irritancy, contact and leakage. CONCLUSIONS: The results demonstrate that the Chamber X offers a cost effective patch test system comparable to IQ and Finn chambers in terms of safety, adhesion, leakage and occlusivity.

Pruritic porokeratotic peno-scrotal plaques: porokeratosis or porokeratotic epidermal reaction pattern? A report of 10 cases.

BACKGROUND: Porokeratosis restricted to the genital region is rare with few cases described in the literature. Cases of porokeratosis restricted to the genital region are similar to plaque type of porokeratosis of Mibelli seen elsewhere on the body. We encountered 10 young males with pruritic plaques restricted to the peno-scrotal region, which clinically were not diagnosed as porokeratosis, but on biopsy revealed multiple cornoid lamellae, some of which were seen to arise from eccrine and follicular structures. AIMS: The aim of this study is to study lesions restricted to the peno-scrotal region in males, which on biopsy showed cornoid lamellae suggestive of porokeratosis. METHODS: Retrospective analysis of available data of patients who were rendered a histological diagnosis of genital porokeratosis. The database consisted of biopsies received in private consultation by the first author in the period January 2000 to March 2013. RESULTS: Ten young men, 8 in their third decade, presented with pruritic plaques restricted to the peno-scrotal region of variable duration. The lesions were well-demarcated on the penis, but ill-defined with a rough granular surface on the scrotum. None of patients were diagnosed clinically as porokeratosis. The lesions were poorly responsive to topical steroid/antifungal treatment, but two patients showed partial improvement with oral isotretinoin. Biopsy in nine patients revealed multiple cornoid lamellae involving epidermis (6) and adnexal structures (3). One patient had a single cornoid lamella. CONCLUSION: The clinical and histological presentation of these patients is different from typical genital porokeratosis described in the literature and we postulate that these patients have an unusual porokeratotic reaction pattern of the epidermis with multiple cornoid lamellae.

Alopecia mucinosa responding to antileprosy treatment: are we missing something?

Three cases with single lesion of Alopecia mucinosa (follicular mucinosis) were treated with antileprosy treatment and showed rapid and complete resolution of the lesions with no recurrence on extended follow-up. Two children, a boy aged 14 years and a girl aged 12 years presented themselves, each, with a single hypopigmented, hypoesthetic patch on the face. Clinically leprosy was suspected, however, skin biopsy from both patients revealed follicular mucinosis as the only pathological finding, without any granulomas. Based on clinical suspicion both were started on multi drug therapy (MDT) for leprosy with complete resolution of the lesions. The third case, male, aged 22 years presented with a single erythematous, hypoesthetic plaque on the forehead. This lesion had been diagnosed as follicular mucinosis with folliculo-tropic mycosis fungoides, in the USA. He too responded completely within 3 months with rifampicin, ofloxacin, minocycline (ROM) treatment, which was given once monthly for a total of 6 months and remains free of disease since the past 1 year. Follicular mucinosis as the only pathology may be seen in facial lesions of clinically suspected leprosy in children and young adults. Based on histological findings these cannot be diagnosed as leprosy and will be considered as Alopecia mucinosa. These lesions, however, are always single and show rapid and complete response to antileprosy treatment. The authors suggest that in regions endemic for leprosy, such as India, single lesion Alopecia mucinosa on the face in children and young adults should be given antileprosy treatment.

Interface dermatitis.

Interface dermatitis includes diseases in which the primary pathology involves the dermo-epidermal junction. The salient histological findings include basal cell vacuolization, apoptotic keratinocytes (colloid or Civatte bodies), and obscuring of the dermo-epidermal junction by inflammatory cells. Secondary changes of the epidermis and papillary dermis along with type, distribution and density of inflammatory cells are used for the differential diagnoses of the various diseases that exhibit interface changes. Lupus erythematosus, dermatomyositis, lichen planus, graft versus host disease, erythema multiforme, fixed drug eruptions, lichen striatus, and pityriasis lichenoides are considered major interface diseases. Several other diseases (inflammatory, infective, and neoplastic) may show interface changes.

Clues to histopathological diagnosis of treated leprosy.

BACKGROUND: Current recommendations for multidrug therapy (MDT) of leprosy follow a fixed duration of treatment regardless of clearance of skin lesions or presence or absence of acid-fast bacilli in the skin. A fairly high percentage of patients with leprosy who complete recommended duration of multi-drug therapy are left with residual skin lesions which are a great source of anxiety to the patient and the family. A small percentage of patients go on to develop new lesions after completion of treatment which may be either late reactions or relapse. Many such patients undergo skin biopsy to assess 'activity' of the disease. Hardly any literature exists on the histological findings in biopsies taken from patients who have completed MDT. MATERIALS AND METHODS: This article describes histomorphological findings in patients with treated leprosy who underwent skin biopsies after completion of MDT because they either had persistent lesions or developed new lesions on follow-up. RESULTS: Histology of treated leprosy may show findings that are diagnostic for leprosy (histology active) or findings that by themselves are not diagnostic for leprosy (histology inactive) but may be used as clues in confirming that the persistent skin lesions are histologically inactive and need no further treatment. These findings may be divided into 1. Epidermal findings, 2. Alterations in dermal stroma, and 3. Morphological characteristics of the dermal inflammatory infiltrate. CONCLUSION: Awareness of histomorphological changes that occur in skin lesions of leprosy after completion of treatment can aid the pathologist to determine whether the lesions are active or inactive histologically and assist the clinician to convince the patient that his disease is inactive and does not need further treatment.

Idiopathic eruptive macular pigmentation or acanthosis nigricans?

A 21-year-old male presented with numerous asymptomatic dark brown to brown black pigmented macules and barely raised plaques on the face, trunk and extremities, developing over a period of 2 year. The surface of most lesions had a velvety raised surface similar in appearance to acanthosis nigricans and a biopsy from one of the lesions showed pigmented papillomatosis. This case fulfils all the criteria for idiopathic eruptive macular pigmentation (IEMP) with papillomatosis, and the clinical appearance of acanthosis nigricans in most lesions suggests that IEMP is a form of eruptive acanthosis nigricans.