[Atypical scleromyxedema with a granulomatous histological pattern and delayed sclerosis]. / Scléromyxœdème atypique, avec aspect histologique granulomateux et apparition retardée de la sclérose.

BACKGROUND: Papular mucinosis is characterised by primary mucin deposition in the dermis. The classification distinguishes between the localised form and the systemic form, which alone can result in complications, but this classification occasionally proves to be inadequate. Herein we report the progression of papular mucinosis, initially atypical due to the absence of cutaneous sclerosis and of misleading granulomatous histological features, which subsequently developed into characteristic scleromyxedema. PATIENTS AND METHODS: A 55-year-old male developed a rash comprising countless acral papules. Several biopsies were necessary before a diagnosis of papular mucinosis was made, due to the initial granulomatous appearance at histology. Tests showed monoclonal immunoglobulin of indeterminate significance, but, due to the absence of cutaneous sclerosis, we were able to conclude on typical localised papular mucinosis. Two years later, extensive sclerotic induration of the skin appeared and the diagnosis was modified to one of scleromyxedema. Treatment with intravenous immunoglobulins was given and proved efficacious, but relapse occurred on discontinuation of the therapy. DISCUSSION: Papular mucinosis is a rare disease of unknown physiopathology. The disease classification distinguishes between the localised and systemic forms, but it occasionally proves to be inadequate. Our case suggests a continuum between the localised and systemic forms of the disease. Further, the initial biopsies of acral papules in our patient had a misleading granulomatous appearance, as has been reported numerous times for the systemic forms. This granulomatous histological variant thus appears to constitute a diagnostic criterion for scleromyxedema.

[Ganulomatosis with polyangiitis revealed by a cutaneous ulceration mimicking a pyoderma gangrenosum: Two patients]. / Granulomatose avec polyangéite révélée par une ulcération cutanée mimant un pyoderma gangrenosum : à propos de 2 cas.

INTRODUCTION: Granulomatosis with polyangiitis is a systemic and necrotizing vasculitis, and cutaneous involvement is uncommon. We report two cases of skin ulceration mimicking a pyoderma gangrenosum, and revealing granulomatosis with polyangiitis. CASE REPORTS: We report two patients who presented with atypical cutaneous ulcerations, with a chronic course. These wounds were large ulcerations with abrupt edges, with purulent and hemorrhagic exudates. The first hypothesis was a pyoderma gangrenosum, but the biopsies were not specific. New biopsies performed distant from the edges showed a necrotizing vasculitis associated with giant cells granuloma, typical from granulomatosis with polyangiitis. CONCLUSION: Cutaneous manifestations are uncommon in granulomatosis with polyangiitis, and can be misleading as they may precede the systemic symptoms. We report two cases of granulomatosis with polyangiitis revealed by cutaneous symptoms mimicking a pyoderma gangrenosum. Repetition of the skin biopsies were necessary to obtain the diagnosis.

[A second case of white lentiginosis]. / Un second cas de lentiginose blanche.

INTRODUCTION: White lentiginosis is characterized by white guttate macules with a typical histological structure: lentiginosis hyperplasia with hypopigmentation. OBSERVATION: A 23-year-old woman, from Algeria, presented with white macules from 5 years ago. Few women in her family had the same trouble. Cutaneous examination revealed diffused white little macules. Histological examination showed a lentiginous hyperplasia of the epidermis, with elongated club-shaped rete ridges and an unusual loss of pigmentation. DISCUSSION: Different diagnosis should be discussed with disseminated leucoderma macules. Nevertheless, the only one which corresponds with our case is the white lentiginosis. To our knowledge, it is the first case described since Grosshans et al. in 1994. Our patient is a descendant of the original case's family. This new case appears to confirm the genetic origin of the disorder and suggests autosomal dominant or X-linked inheritance.

[Fibrous papules of the face: a retrospective anatomoclinical study of 283 cases]. / Papules fibreuses du visage: une étude anatomo-clinique rétrospective de 283 cas.

BACKGROUND: Fibrous papules of the face are frequent benign lesions seen in the nasal and perinasal region. Their clinical aspect is indistinct and the histological signs are sometimes mild or possibly misleading in the case of atypical forms. We carried out a retrospective study of 283 fibrous papules diagnosed at our histology laboratory. The goal of this study was to characterize this type of frequent but occasionally unrecognized lesion. PATIENTS AND METHODS: We performed a retrospective study of fibrous papules of the face diagnosed in the dermatopathology laboratory of our dermatology centre between January 2002 and December 2011. The study concerned the clinical information noted in the examination request and the morphological abnormalities seen at optical microscopy. An immunohistological study of factor XIIIa was performed in selected cases. RESULTS: The fibrous papules of the face came from 129 men and 154 women aged between 18 and 90 years (mean: 46 years). Two hundred and thirty-seven (83.7%) lesions were taken from the nasal region and none were taken from anywhere other than the face. The clinically mentioned diagnoses varied. A diagnosis of fibrous papule of the face was stated in 42% of cases, and the main differential diagnoses were nevus (stated in 34% of cases) and basal cell carcinoma (stated in 14% of cases). The fibrous papules were classic in 85.5% of cases. We observed 6 variants of fibrous papule: hypercellular, inflammatory, pleomorphic, pigmented, clear-cell and granular-cell types. Immunohistochemistry of factor XIIIa was positive in all cases except clear-cell fibrous papules. DISCUSSION: This study shows that despite their frequency, these lesions often go unrecognized, since the hypothesis of a fibrous papule of the face was mentioned in fewer than 50% of cases at the time of biopsy. Diagnosis is often made by the histopathologist, who may be misled by some rare types. The principal differential diagnoses are nevus and basal cell carcinoma, thus warranting methodical histological analysis of all pieces.

[Atypical papular mucinosis with initial histological findings evocative of granuloma annulare]. / Mucinose papuleuse atypique avec aspect histologique initial de granulome annulaire.

BACKGROUND: Papular mucinosis is characterized by primary reticular dermal mucin deposition in the absence of any thyroid abnormalities. There is extensive clinical variety in this disease, from benign localized forms to generalized forms, on occasion lethal. The current classification was established in 1991 in order to allow identification of these generalized forms of the disease and help ensure a better therapeutic approach. We report the case of a patient with atypical papular mucinosis having initial histological features consistent with granuloma annulare. PATIENTS AND METHODS: A 55-year-old man consulted for a papular eruption of the hands. A diagnosis of granuloma annulare was initially made because of misleading histological findings. However, the eruption spread to the inner thighs and the hypogastric area despite dermocorticoid therapy. Laboratory evaluation showed a monoclonal gammopathy. Further biopsies revealed diffuse dermal mucin deposits. We finally concluded on atypical localized papular mucinosis. DISCUSSION: Papular mucinosis is a rare disease and its physiopathology remains to be elucidated. The diagnostic criteria are sometimes inadequate, and its classification includes both atypical and intermediate forms. Our case belongs to the latter class because of its extensive and unusual topography, and its association with a monoclonal gammopathy. Although granuloma annulare is not a classical differential diagnosis, two other cases with similar histological findings have already been published.