An unusually severe case of dermatosis neglecta: a diagnostic challenge.

Dermatosis neglecta is a condition secondary to lack of cleanliness, characterized by the formation of hyperkeratotic plaques located in a particular region of the body, usually due to a disability, and it is considered a diagnostic challenge because it can mimic other entities. We present the case of an 18-year-old woman with a 2-month history of progressive brown verrucous plaque in her face. Our first impression was seborrheic dermatitis and she was treated with facial cleanser soap and topic hydrocortisone 1%. One month later, she arrived with new lesions. Because of this our diagnosis changed to Darier's disease versus seborrheic pemphigus versus foliaceus pemphigus. Histopathology and immunofluorescence studies were compatible with seborrheic dermatitis. The patient complained of depression and social withdrawal and denied facial cleansing. Facial cleansing was performed during consultation presenting resolution of the lesions, which confirmed the diagnosis of dermatosis neglecta. Dermatosis neglecta is a disease that can be frequently misdiagnosed, since it has many differential diagnoses, such as hyperkeratotic syndromes. It should be recognized early and aggressive diagnostic and therapeutic methods should be avoided.

Introduction

Dermatosis neglecta is a condition secondary to lack of cleanliness, characterized by the formation of hyperkeratotic plaques located in a specific region of the body, usually due to a psychiatric condition, physical disability or neurological deficit. It is considered a diagnostic challenge [1] because it can mimic other entities such as hyperkeratotic syndromes or hyperchromic lesions [2]. We present a patient in whom it was difficult to establish the diagnosis.

Case Presentation

An 18-year-old woman presented with a 2-month history of progressive brown verrucous plaque on the face, previously treated with no improvement (fig. 1). Our first impression was seborrheic dermatitis and we treated her with facial cleanser soap, topical hydrocortisone 1% b.i.d. per 3 days and then q.d. per 3 days. One month later she arrived with new lesions, therefore our diagnosis changed to Darier's disease versus seborrheic pemphigus versus foliaceus pemphigus. Biopsy with histopathological and immunofluorescence studies were ordered with results being compatible with seborrheic dermatitis.

Fig. 1

Patient at first visit.

We performed a new patient interview and discovered depression secondary to her pathology as well as social withdrawal. In addition, she reported not following instructions and denied any facial cleansing. We performed face cleaning during the consultation. The lesions disappeared and this confirmed the diagnosis of dermatosis neglecta (fig. 2).

Fig. 2

Patient after facial cleansing.

Discussion

Dermatitis neglecta or unwashed dermatosis was first described in 1995 by Poskitt et al. [3] as a condition secondary to the accumulation of sebum, sweat, corneocytes and bacteria forming a hyperkeratotic plaque [4]; later, in 1999, Ruiz-Maldonado et al. [5] changed the name to dermatosis neglecta due to the lack of inflammation.

Dermatosis neglecta is sometimes underdiagnosed because it is asymptomatic [2]. If affects all age groups and both sexes and clinically presents as hyperkeratotic plaques with cornflake-like scales with an evolution of 2–4 months; the lesions disappear with proper washing of the affected area [6, 7]. The diagnosis is clinical and lesions can be identified by removing the crusts with isopropyl alcohol [1]. A biopsy is usually not necessary and orthokeratotic hyperkeratosis, papillomatosis and mild acanthosis can be seen without an inflammatory infiltrate [8, 9].

The main differential diagnoses are terra firma-forme dermatosis, confluent reticulated papillomatosis of Gourgeot and Carteaud, acanthosis nigricans, verrucous nevi, vagabond's disease, and genetic disorders such as Darier's disease and X-linked ichthyosis [1, 8]. Terra firma-forme dermatosis is clinically very similar to dermatosis neglecta, but unlike dermatosis neglecta, it has a history of good hygiene and responds only to cleaning with isopropyl alcohol 70% [2, 6, 8]. Dermatitis artefacta presents as self-inflicted injuries or injuries worsened by the patient, while dermatosis neglecta is an act of omission of cleaning by the patient either intentionally or unconsciously [8]. A diagnostic algorithm can be used as a tool when confronted with a difficult pathology [10] (fig. 3). Treatment is based on reinforcing hygiene measures and using keratolytic agents such as urea, retinoic acid, glycolic acid, lactic acid or salicylic acid [1, 7, 11].

Fig. 3

Algorithm for dirt-like lesions characterized by a pigmented and dirty appearance (based on [10]).

Conclusion

Dermatosis neglecta is a disease that can be frequently misdiagnosed, since it has many differential diagnoses which include hyperkeratotic syndromes. It should be recognized early to avoid aggressive diagnostic and therapeutic procedures.

Disclosure Statement

The authors have no conflict of interest to declare. There were no funding sources.