Lumír Pock

Lichen planus pigmentosus–inversus

We examined seven patients with lichen planus pigmentosus (LPP) clinically and microscopically. Clinically, all patients had a striking predominance of lesions in an intertriginous location, with most of them in the axillae. Microscopically, two biopsies were of significance. Except for the regressive lichen planus, which is usual in LPP, the active inflammatory phase was also present. In these biopsies the very intensive hydropic degeneration of basal keratinocytes was combined with the absence of compensatory increased proliferation of keratinocytes, i.e. without acanthosis. The short duration of this process probably led to the quick transformation into a long noninflammatory regressive phase with incontinence of the pigment. These specific morphogenetic dynamics are possibly why most of the morphs of LPP present as brown, non‐pruritic, small inflammatory macules. Because of the highly characteristic inverse location of the lesions in our patients we propose the designation LPP–inversus for this variant of the disease.

Dermatoscopy of pigmented actinic keratosis – A striking similarity to lentigo maligna

Background  Pigmented actinic keratosis (PAK) resembles lentigo maligna (LM) clinically and histopathologically in some cases.

Crystal storing histiocytosis of the skin associated with multiple myeloma

Background  Crystal storing histiocytosis (CSH) imitating rhabdomyom is a very rare disease entity involving different tissues. The skin is involved in extremely rare cases.

Pimecrolimus is effective in Fox-Fordyce disease.

International Journal of Dermatology 2006, 45, 1134 –1135

Annular elastolytic giant cell granuloma causes an irreversible disappearance of the elastic fibres

A 67‐year‐old man presented with grouped red papules with a smooth surface coalescing to relatively well‐demarcated plaques on his left thigh, in the axillae and on the lateral parts of the trunk. The plaques were growing slowly, and the older ones had a frilled surface. A skin biopsy showed a zone of disappearance of the elastic fibres with a rim of giant multinuclear cells with fragments of the elastic fibres in their cytoplasm. This finding is typical of annular elastolytic giant cell granuloma (AEGCG). After corticosteroid therapy, the inflammation resolved, causing the frilled surface of the lesions due to the disappearance of the elastic fibres. Remission of the skin lesions lasted for 1.5 years. A second skin biopsy taken from the site of the previous lesion showed the absence of the elastic fibres, thus their phagocytosis was irreversible.

Verrucous form of chilblain lupus erythematosus

A 45‐year‐old woman had symmetrical livid plaques with yellowish hyperkeratoses for 5 years, which progressed on to the fingers and toes and on the soles of the feet. Two years later creamy, whitish areas and maceration appeared on the buccal mucosa and the lips. A skin biopsy revealed massive collagen hyaline degeneration in the perivascular area, hyperkeratosis and hypergranulosis, small lymphocyte infiltrates with several melanophages and extravasates of erythrocytes in the upper corium in perivascular areas and hydropic degeneration of basal keratinocytes. The findings using direct immunofluorescence were compatible with lupus erythematosus (LE). Laboratory investigation showed a slight leucopenia and thrombopenia, a slightly elevated erythrocyte sedimentation rate, hypocomplementaemia C3 and C4, a high titre of rheumatoid factor and antinuclear antibodies positivity of extractable nuclear antigen. The results reflected probably the development of a systemic form of the disease. The patient was successfully managed by methylprednisolone and hydroxychloroquine. After 1 year of therapy, a new skin biopsy revealed a substantial reduction of hyperkeratosis and hyaline degeneration of collagen tissue in the perivascular areas. The combination of the extensive hyperkeratosis and hyalinization thus seems to be features of the long‐lasting, untreated lesions in chilblain LE.

SYSTEMATIZED PROGRADIENT MULTIPLE COMBINED MELANOCYTIC AND BLUE NEVUS

A 44-year-old woman was diagnosed as having unilateral multiple progradient pigmented macules and papules of the upper extremity and adjacent part of the back. Microscopically increased amounts of melanin and melanocytes in the basal layer of the epidermis in the early developmental stage of macules were seen. Combinations of blue nevus with junctional or compound nevus or with a simple proliferation of melanocytes in the epidermis were present in the papules. The question of prognosis is discussed.

Agminated histiocytomas in a 23-year-old patient

A 23‐year‐old patient presented with a group of 17 reddish‐brown papules and nodules on the left shoulder lasting for 4 years. The histopathologic examination after a punch biopsy was repetitively consistent with the diagnosis of fibrous histiocytoma (FH). We use the term agminate histiocytomas for the first time to stress the presence of grouped lesions in one skin segment. Intralesional corticosteroids and cryotherapy were partially successful in this patient.

Segmental Pigmented Purpura

Abstract: A segmental distribution of pigmented purpura (PP) is rare. Our patient presented at 5 years of age with red‐brown petechial macules and telangiectases on her chest, the inner and dorsal aspects of the right upper extremity, and the dorsal aspect of the hand, which had developed slowly over the previous 7 months. The lesions were intermittently pruritic. A biopsy from the right arm revealed discrete hydropic degeneration of the basal layer of the epidermis, dilation of the blood vessels, numerous extravasated erythrocytes and lymphocytes, and lymphocytes in the lower part of the epidermis. The skin disease resolved almost completely within 18 months. The general physical examination revealed an open foramen ovale, slight hypogammaglobulinemia (G and A), and enhanced fragility of the blood vessels.